Omphalitis

Mohamed Fahmy Umbilicus and Umbilical Cord Contents Part I Introduction 1 Nomenclature and Synonyms of the Umbilicus . . . . . . . . . . . . . 3 2 Definition . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Reference . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 5 5 3 Origin of the Name of Umbilicus . . . . . . . . . . . . . . . . . . . . . . . . . 3.1 Origin of Omphalos . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3.2 The Omphalos in Delphi . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3.3 Agricultural Origin of the Name of Umbilicus . . . . . . . . . . . References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 7 8 9 10 11 4 Umbilicus in Different Languages . . . . . . . . . . . . . . . . . . . . . . . . 13 5 Names Related to Umbilicus . . . . . . . . . . . . . . . . . . . . . . . . . . . . 15 6 Other Uses of the Name of Umbilicus . . . . . . . . . . . . . . . . . . . . . 17 7 Umbilicus of Plants . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 19 21 8 Animal’s Navel . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 8.1 Fate of Animal Cord and How They Are Cut . . . . . . . . . . . . References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 23 23 26 9 Umbilicus in History and Its Religious Background . . . . . . . . . 9.1 Adam and Eve Umbilicus . . . . . . . . . . . . . . . . . . . . . . . . . . . 9.2 Umbilicus and Umbilical Cord in Different Cultures . . . . . . 9.3 Umbilical Cord and Tradition . . . . . . . . . . . . . . . . . . . . . . . . 9.4 Spiritual Facts About Umbilicus and Umbilical Cord . . . . . . References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 27 27 29 32 36 40 ix x Contents Part II Umbilical Cord 10 Embryology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 43 45 11 Anatomy of the Umbilical Cord. . . . . . . . . . . . . . . . . . . . . . . . . . 11.1 Cord Length . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 11.2 Long Umbilical Cord . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 11.3 Abnormal Length and Nuchal Cord . . . . . . . . . . . . . . . . . . . 11.4 Conditions Associated with Short Cord. . . . . . . . . . . . . . . . 11.5 Innervation of Umbilical Cord . . . . . . . . . . . . . . . . . . . . . . . References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 47 47 47 48 49 54 55 12 Physiology, Ultrastructure, Ultrasonography and Pharmacology of the Umbilical Cord . . . . . . . . . . . . . . . . . 12.1 How the Human Umbilical Cord Works? . . . . . . . . . . . . . . 12.2 Ultrastructure of Umbilical Cord . . . . . . . . . . . . . . . . . . . . . 12.2.1 Umbilical Cord Design . . . . . . . . . . . . . . . . . . . . . 12.2.2 Spiral Turns of the Cord . . . . . . . . . . . . . . . . . . . . . 12.3 Contractility of Umbilical Vessels . . . . . . . . . . . . . . . . . . . . References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 57 57 58 58 59 59 62 13 Congenital Anomalies of the Umbilical Cord. . . . . . . . . . . . . . . 13.1 Placental Attachment (Site of Cord Insertion) . . . . . . . . . . . 13.2 Velamentous Cord Insertion. . . . . . . . . . . . . . . . . . . . . . . . . 13.3 Furcate Cord Insertion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 13.4 Single Umbilical Artery . . . . . . . . . . . . . . . . . . . . . . . . . . . . References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 63 63 64 65 65 65 14 Umbilical Cord Cysts . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 14.1 Differential Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 14.2 Pathology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 14.3 Associated Anomalies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 14.4 Radiographic Features . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 14.5 Treatment and Prognosis . . . . . . . . . . . . . . . . . . . . . . . . . . . References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 67 68 68 70 70 70 71 15 Umbilical Cord Knots . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 15.1 False Knots. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 73 74 74 16 Umbilical Cord Prolapse . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 75 77 17 Umbilical Cord Tumors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 17.1 Umbilical Cord Teratoma . . . . . . . . . . . . . . . . . . . . . . . . . . 17.2 Haemangioma of Umbilical Cord . . . . . . . . . . . . . . . . . . . . 17.3 Umbilical Cord Haematoma . . . . . . . . . . . . . . . . . . . . . . . . References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 79 79 81 84 85 18 Rare Anomalies of the Umbilical Cord . . . . . . . . . . . . . . . . . . . . 18.1 Thrombosis of Umbilical Vessels . . . . . . . . . . . . . . . . . . . . 87 88 Contents xi 18.2 Strictures or Coarctation of the Umbilical Cord . . . . . . . . . 18.3 Umbilical Vein Varix . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 18.4 Umbilical Artery Aneurysm . . . . . . . . . . . . . . . . . . . . . . . . References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Part III 88 89 89 90 Normal Umbilicus 19 Embryology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 19.1 Separation of the Umbilical Cord . . . . . . . . . . . . . . . . . . . . 19.2 Delayed Separation of the Umbilical Cord . . . . . . . . . . . . . References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 20 Anatomy and Physiology of the Umbilicus . . . . . . . . . . . . . . . . . 97 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 100 21 Umbilical Position . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 101 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 103 22 Umbilicus Types and Shapes . . . . . . . . . . . . . . . . . . . . . . . . . . . . 105 22.1 Ugly Umbilical Scar . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 107 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 108 23 Umbilical Landmark . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 109 24 Uses of the Umbilicus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 111 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 113 25 Umbilical Gaze . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 115 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 116 Part IV 95 95 96 96 Acquired Umbilical Disorders 26 Omphalitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 26.1 Neonatal Omphalitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 26.2 Phlegmonous Omphalitis . . . . . . . . . . . . . . . . . . . . . . . . . . . 26.3 Differential Diagnoses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 26.4 Investigations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 26.5 Complications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 26.5.1 Necrotizing Fasciitis (NF) . . . . . . . . . . . . . . . . . . . 26.6 Treatment of Omphalitis . . . . . . . . . . . . . . . . . . . . . . . . . . . 26.7 Omphalitis in Adult . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 26.8 Navel Piercing Infection . . . . . . . . . . . . . . . . . . . . . . . . . . . 26.9 Management of Omphalitis in Adults . . . . . . . . . . . . . . . . . 26.10 Rare Types of Omphalitis . . . . . . . . . . . . . . . . . . . . . . . . . . 26.10.1 Umbilical Tetanus Neonatorum . . . . . . . . . . . . . . . References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 27 Umbilical Granuloma (UG) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 133 27.1 Clinically . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 135 119 120 120 123 123 124 124 125 127 128 129 129 129 130 xii Contents 27.1.1 Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 27.2 Umbilical Granuloma Beyond Neonatal Age . . . . . . . . . . . 27.3 Umbilical Lesions Looks Like UG . . . . . . . . . . . . . . . . . . . References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 137 141 141 142 Umbilical Hernia (UH) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 28.1 Infantile Umbilical Hernia . . . . . . . . . . . . . . . . . . . . . . . . . . 28.2 Immediate Causes Lead to Development of Umbilical Hernia. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 28.3 Syndromes Associated with a High Incidence of UH . . . . . 28.4 Pathophysiology of UH . . . . . . . . . . . . . . . . . . . . . . . . . . . . 28.5 Clinical Picture of UH . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 28.6 Complications of UH . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 28.7 Management. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 28.8 Proboscoid Umbilical Hernia (PUH) . . . . . . . . . . . . . . . . . . 28.9 Acquired Umbilical Hernia . . . . . . . . . . . . . . . . . . . . . . . . . 28.9.1 Serous Umbilical Hernia . . . . . . . . . . . . . . . . . . . . 28.10 Recurrent Umbilical Hernias . . . . . . . . . . . . . . . . . . . . . . . . References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 145 146 150 150 151 152 152 153 156 158 158 160 161 29 Umbilical Polyp (UP) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 29.1 Omphalomesenteric Duct Polyp . . . . . . . . . . . . . . . . . . . . . 29.2 Umbilical Polyp Originating from Urachal Remnants . . . . 29.3 Ectopic Mucosa Polyp . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 29.4 Fibrous Umbilical Polyp (FUP) . . . . . . . . . . . . . . . . . . . . . . References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 163 166 167 168 169 170 30 Umbilical Neoplasm . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 30.1 Umbilical Endometriosis (UEM) . . . . . . . . . . . . . . . . . . . . . 30.2 Umbilical Haemangioma . . . . . . . . . . . . . . . . . . . . . . . . . . . 30.3 Epithelial Inclusion Dermoid Cysts Discussed in Chap. 31 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 30.4 Melanocytic Nevi . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 30.5 Benign Connective Tissue Growths . . . . . . . . . . . . . . . . . . . 30.5.1 Fibroepithelial Papillomas (Umbilical Warts) . . . . 30.5.2 Dermatofibroma . . . . . . . . . . . . . . . . . . . . . . . . . . . 30.6 Umbilical Carcinoma (B. Malignant Umbilical Tumors) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 30.6.1 Primary Umbilical Carcinoma . . . . . . . . . . . . . . . . 30.6.2 Umbilical Melanoma: Navel Melanoma . . . . . . . . 30.6.3 Secondary Umbilical Carcinomas . . . . . . . . . . . . . References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 171 171 175 Rare Umbilical Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 31.1 Absent Umbilicus. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 31.1.1 Congenitally Absent Umbilicus . . . . . . . . . . . . . . . 31.1.2 Acquired Absent Umbilicus . . . . . . . . . . . . . . . . . . 31.2 Omphalith . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 31.3 Belly-Button Lint . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 185 185 185 187 188 190 28 31 177 177 177 177 178 178 178 179 180 182 Contents xiii 31.4 Umbilical Dermoid and Epidermoid Cyst . . . . . . . . . . . . . . 31.5 Umbilical Pilonidal Sinus (PNS) . . . . . . . . . . . . . . . . . . . . . 31.6 Umbilical Keloid . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Part V 191 192 193 195 Umbilical Disorders: Congenital Anomalies of Umbilicus 32 Congenital Hernia of Umbilical Cord (CHUC) . . . . . . . . . . . . . 32.1 Cardinal Signs for Diagnosis of CHUC . . . . . . . . . . . . . . . . 32.2 Umbilicus Cutis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 199 201 203 204 33 Gastroschisis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 33.1 Gastroschisis Variants . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 33.2 Prenatal Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 33.3 Complications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 33.4 Outcome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 33.5 Management. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 33.6 Surgical Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 207 210 210 212 212 213 213 215 34 Examophalos (Omphalocoele) . . . . . . . . . . . . . . . . . . . . . . . . . . . 34.1 Etiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 34.2 Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 34.3 Management. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 217 217 221 225 227 35 Urachal Anomalies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 35.1 Embryology of Urachus . . . . . . . . . . . . . . . . . . . . . . . . . . . . 35.2 Ontogenetic and Structural Study of the Urachus . . . . . . . . 35.3 Patent Urachus (PU) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 35.3.1 Historical Background . . . . . . . . . . . . . . . . . . . . . . 35.3.2 Incidence . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 35.3.3 Pathogenesis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 35.3.4 Patent Urachus and Fetal Obstructive Uropathy . . 35.4 Urachal Cyst. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 35.5 Urachal Sinus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 35.6 Urachal Abscess . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 35.7 Urachal Diverticulum (UD) ‘Vesicourachal Diverticulum’ . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 35.8 Urachal Neoplasm . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 35.9 Absent Urachus: ‘Urachal Agenesis’ . . . . . . . . . . . . . . . . . . References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 229 231 232 234 234 234 235 235 241 245 246 Vitellointestinal Duct Anomalies . . . . . . . . . . . . . . . . . . . . . . . . . 36.1 Meckel’s Diverticulum (MD). . . . . . . . . . . . . . . . . . . . . . . . 36.2 Patent Vitellointestinal Duct . . . . . . . . . . . . . . . . . . . . . . . . 36.3 Vitellointestinal Sinus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 36.4 Vitellointestinal Cyst (Vitelline Duct Cysts) . . . . . . . . . . . . 36.5 Vitellointestinal Fibrous band . . . . . . . . . . . . . . . . . . . . . . . 253 256 257 260 260 260 36 246 248 250 251 xiv Contents 36.6 Intestinal Prolapse . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 260 36.7 Congenital Umbilical Appendix (Appendico-Umbilical Fistula) . . . . . . . . . . . . . . . . . . . . . . 261 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 263 Part IV Acquired Umbilical Disorders 26 Omphalitis Omphalitis will be discussed under the following headings: • Neonatal omphalitis • Adult omphalitis – Bacterial – Non bacterial • Rare types of omphalitis Nomenclature: Belly Button Infection of Newborns. Definition: Omphalitis term usually applied for the bacterial neonatal umbilical stump infection, but other types of specific and non specific umbilical infection are roughly called omphalitis. It is an infection of the umbilicus and/or surrounding tissues, and it is predominantly a disease of the neonate, characterized by discharge from the umbilical cord stump with surrounding induration, erythema, and tenderness (Fig. 26.1). Omphalitis is not only human-being disease, but it occurs also and commonly in the newborn calf, and chickens, in such cases omphalitis is a condition characterized by infected yolk sacs, often accompanied by unhealed navels in young fowl, and the resulting septicaemia contribute to perinatal mortality in several animal species (Fig. 26.2). Funisitis (umbilical arteritis, or vasculitis) is an intrauterine inflammation of the connective tissue of the umbilical cord, it is typically preceded by vasculitis of the umbilical artery or veins and may be the result of chorioamnionitis, which may end with abortion. Funisitis may be detected either antenatally or immediately after Fig. 26.1 Early bacterial omphalitis of neonate Fig. 26.2 Animal omphalitis, infected umbilical stump of a calf birth, this usually results in a wet, foul-smelling umbilical stump and is usually caused by inflammation driven by group A strep (Fig. 26.3). © Springer International Publishing AG 2018 M. Fahmy, Umbilicus and Umbilical Cord, https://doi.org/10.1007/978-3-319-62383-2_26 119 120 26 Omphalitis Fig. 26.3 Funisitis characterized by umbilical arterial infiltration of neutrophils 26.1 Neonatal Omphalitis (Fig. 26.1) Catarrhal omphalitis: It is also known as the ‘weeping navel’. The clinical signs of catarrhal omphalitis are: • Serous (transparent) discharge from the umbilical wound. • Slow healing of the wound. • Slight reddening of the umbilical ring. • Normal body temperature. Sometimes the wound covered with a thick bloody crust under which the discharge accumulates. In cases when catarrhal omphalitis prolongs for more than 2 weeks (with treatment) it can develop into belly button fungus. Infants with large body mass at birth and those who have a thick cord and a broad umbilical ring are prone to the development of belly button fungal infection. If left untreated, catarrhal omphalitis develops into purulent omphalitis. If the infection spreads even further, the inflammation goes deeper into the umbilical tissue, which leads to the development of phlegmonous omphalitis. 26.2 Phlegmonous Omphalitis (Fig. 26.4) Phlegmon is a spreading diffuse inflammatory process with formation of purulent exudate. The term ‘phlegmon’ (from Greek ‘phlegmone’ means inflammation) mostly refers to a walledoff inflammatory mass. Signs and symptoms: Phlegmonous omphalitis is a bacterial inflammation of the bottom of the umbilical wound, umbilical ring, and of the subcutaneous fat around the umbilical ring. This disease begins much alike catarrhal omphalitis. However, after some days transparent discharge from the wound turns into purulent. The umbilical ring becomes swollen, there is pronounced redness in the umbilical region. The subcutaneous fat becomes dense and starts bulging above the anterior abdominal wall. The skin around the navel is hot. Dilated vessels and sometimes red stripes are seen through the skin. Very often phlegmonous omphalitis is accompanied by an infection of umbilical vessels, which may lead to the development of septicaemia and an infected embolisation may spread to the liver, peritoneal or retroperitoneal spaces. 26.2 Phlegmonous Omphalitis 121 hospital study of newborns who were routinely bathed with hexachlorophene, the 6-year incidence of cord infections was 0.5% in newborns of normal weight and 2.08% in those born prematurely [4]. Sex: There is no sex predilection has been reported, although males may have a worse prognosis than females, also incidence does not appear to have any racial or ethnic predilection. Age: In full-term infants, the mean age at onset is 5–9 days. In preterm infants, the mean age at onset is 3–5 days. Risk Factors: Identified risk factors for neonatal omphalitis may be simply classified to: Fig. 26.4 Phlegmonous omphalitis, with purulent discharge beneath umbilical stump Incidence: The current incidence of omphalitis in the United States is somewhere around 0.5% per year overall, the incidence rate for European countries falls between 0.2 and 0.7% [1]. But the incidence is greater in developing countries where rates are reported to be as high as 40%, although lacking of precise and complete data from many of these countries, the incidence is expected to be more higher and with a wide variance of the presentation and complications [2]. In one study of neonates admitted to an African general paediatric ward, omphalitis accounted for 28% of neonatal admissions. Another hospital study found that, in 47% of infants hospitalized with sepsis, cord infection was the source of the illness, and that 21% of infants admitted for other reasons had omphalitis. A prospective study in urban slums found an incidence for umbilical sepsis of 30/1000 [3]. Many cases are under-reported as babies may be discharged early from hospital and not followed up at home. Cord care after delivery had a crucial role in reducing omphalitis rate, in one large • Maternal factors: – Intrauterine infection, like placental infection (chorioamnionitis) – Uncontrolled mother diabetes • Delivery situation: – Prolonged delivery and early rupture of membranes, nonsterile delivery, and delivery at home. – Unsterile cord cutting • Neonatal factors: – Low birth weight, and prematurity – Patients who have a weakened or deficient immune system. – Neonates who are hospitalized and subject to invasive procedures. – Sick babies with other infections such as blood infection (sepsis) or pneumonia. – Patients who have had umbilical catheters (Fig. 26.5). Umbilical catheters have been used in NICUs for drawing blood samples, measuring blood pressure, and administration of fluid and medications for more than 25 years [5]. Catheterization of the umbilical vein is one of the fastest and easiest methods of gaining access to a deep vein. Complications associated with umbilical catheters include thrombosis; embolism; vasospasm; vessel perforation; haemorrhage; infection; gastrointestinal, renal, and limb tissue damage. Babies with an umbilical catheters may develop omphalitis, due to bacterial colonization, 122 it was found that the umbilical stump is frequently colonised with pathogenic organisms, even when topical antiseptics are regularly applied in the NICU. Also the use of a venous line for infusion of hypertonic or acidic solutions, such as parenteral nutrition solutions, may provide a necrotic focus for abscess formation [6]. Bacteriology: Omphalitis usually caused by S. aureus, group A strep (Streptococcus pyogenes) and occasionally by gram-negative bacilli like Pseudomonas aeruginosa and E. coli, also there is a significant role of anaerobes in this infectious disease specially in necrotizing omphalitis [7]. Approximately three fourths of omphalitis cases are polymicrobial in origin, aerobic bacteria are present in approximately 85% of infections, predominated by Staphylococcus aureus, group A Streptococcus, Escherichia coli, Klebsiella pneumoniae, and Proteus mirabilis [8]. Clinically: It is a rapidly progressive soft tissue infection that arises from the umbilicus and classically spreads along low resistance fascial 26 Omphalitis planes, resulting in tissue necrosis and overwhelming sepsis. Umbilical stump bleeding may occurs with omphalitis as a result of delayed obliteration of the umbilical vessels (Fig. 26.6). Omphalitis most commonly presents with signs of umbilical inflammation (erythema, induration, and swelling), with or without drainage from the umbilical stalk at the first 2 weeks of life (Fig. 26.7). Close observation and a high degree of clinical suspicion for the development of localized fluid collections or necrotizing infection are critical and given the potential need for surgical intervention in cases of neonatal omphalitis. Lack of erythema progression, absence of fever, initial hemodynamic stability and normal activity were falsely reassuring features that delayed operative intervention. Patients with omphalitis may present with purulent umbilical discharge or periumbilical Fig. 26.6 Umbilical stump bleeding as a predictor of omphalitis Fig. 26.5 Omphalitis around an umbilical catheter Fig. 26.7 Omphalitis with signs of inflammation (erythema, induration, and swelling) 26.4 Investigations 123 26.4 Investigations A microbiological swab of the umbilicus should be sent for aerobic and anaerobic cultures. A blood culture should be requasted when appropriate. A blood count with differential for white cell counts may show a neutrohilia (or occasionally a neutropaenia). Other investigations are necessary either to rule out other confusing conditions or to diagnose complications. Fig. 26.8 Omphalitis with purulent discharge cellulitis. Although infections may be associated with retained umbilical cord or ectopic tissue. Cellulitis may become severe within hours and progress to necrotizing fasciitis and generalized sepsis. Even after the stump falls off, the patient with omphalitis may still present with a malodorous umbilicus with a superficial infection of the skin around the area, somewhat like impetigo (Fig. 26.8). 26.3 • A plain abdominal radiograph is useful if necrotising enterocolitis is suspected, in addition, it may reveal intraperitoneal gas in those cases with peritonitis (caused by gas-producing bacteria). • Abdominal ultrasonography is useful in imaging the abdominal wall if a cyst is suspected, it is also helpful in the diagnosis of intraperitoneal, retroperitoneal, and hepatic abscesses. • Doppler ultrasonography is helpful if portal vein thrombosis is suspected. • In few cases with a suspicious of serious complication, a CT scan may be indicated, specially in cases with suspicious of liver affection (Fig. 26.9). Differential Diagnoses The differential diagnoses of omphalitis include: • Umbilical granuloma (visible granuloma at the base of umbilicus) (Chap. 27) • Patent vitellointestinal duct remnants (cystic swelling or fistulous opening with feculent matter discharge) (Chap. 36) • Patent urachus (fistulous opening with urine discharging) or urachal cyst (Chap. 35) • Necrotising enterocolitis (abdominal distention, bilious vomiting, bloody and stools) • General sepsis • Rarely, appendiculo-omphalic anomalies (Sect. 36.7) Fig. 26.9 CT scan showing an abscess in falciform ligament secondary to omphalitis 124 26.5 26 Omphalitis Complications Potential sequelae of omphalitis include necrotizing fasciitis, myonecrosis, septicaemia, portal vein thrombosis, septic embolization; particularly, endocarditis and liver abscess, abdominal complications (e.g., spontaneous bowel evisceration, peritonitis, bowel obstruction, abdominal or retroperitoneal abscess), these sequelae are associated with significant morbidity and mortality. In a retrospective review of 19 neonates and infants treated for major complications of omphalitis: Five (26%) patients presented with spontaneous evisceration of small bowel through the umbilical cicatrix, resulting in intestinal gangrene in one. Necrotizing fasciitis occurred in five (26%) patients involving mainly the scrotum, and in two involving the penis as well. Three (16%) patients had peritonitis, resulting in intraabdominal abscesses in two. Three (16%) had superficial abscesses, two (11%) had hepatic abscesses resulting in extensive destruction of the left lobe in one, and one (5%) developed an adhesive intestinal obstruction [9]. The mortality rate among all infants with omphalitis, including those who develop complications, is estimated at 7–15%. The mortality rate is significantly higher (38–87%) after the development of necrotizing fasciitis or myonecrosis [10]. 26.5.1 Necrotizing Fasciitis (NF) (Fig. 26.10) Necrotizing omphalitis is a rare disease of the newborn with only few cases reported in the literature. This is a florid bacterial infection of the skin, subcutaneous fat, superficial and deep fascia that complicates 8–16% of cases of neonatal omphalitis. It is characterized by rapidly spreading infection and severe systemic toxicity. Necrotizing fasciitis typically involves the abdominal wall but may also involve the scrotum or penis [9]. Necrotizing soft-tissue infections are caused by single or multiple organisms, that lead directly to tissue cell death, enzymatic destruction of supporting connective tissue, and destruction of host Fig. 26.10 Necrotizing fasciitis with early affection of the deep tissues humoral and cellular immune responses to infecting organisms. Certain organisms are well known to invade tissue and proliferate in necrotic areas. Group A Streptococcus, S. aureus, and Clostridium species may elaborate extracellular enzymes and toxins that can damage tissue, and may facilitate movement of organisms through soft-tissue planes, and limit host defences with penetration of systemic antimicrobial agents [11]. Reported survival rates for neonatal necrotizing omphalitis range anywhere from 19 to 40%, reflecting an aggressive disease with significant morbidity and mortality, in most series, omphalitis leading to necrotizing fasciitis is associated with a high mortality rate, up to 80%. Necrotizing fasciitis can also lead to portal venous thrombosis and portal hypertension [12]. Myonecrosis: It generally refers to infectious involvement of muscle in infants with omphalitis, the development of myonecrosis usually depends on conditions that facilitate the growth of anaerobic organisms. These conditions include the presence of necrotic tissue, poor blood supply, foreign material, and established infection by aerobic bacteria such as staphylococci or streptococci, but C. perfringens, in particular, does not replicate under conditions of an oxidation-reduction potential. In infections with mixtures of facultative aerobes and anaerobes, the aerobic organisms use oxygen available in tissue, allowing anaerobic bacterial growth (Fig. 26.11). 26.6 Treatment of Omphalitis Fig. 26.11 Myonecrosis with extensive erosion of umbilicus and abdominal wall above it The toxins produced in the anaerobic environment of necrotic tissue allow rapid spread of organisms through tissue planes. Local spread of toxins extends the area of tissue necrosis, allowing continued growth of organisms and increasing elaboration of toxins. Because of progressive deep tissue destruction and subsequent systemic spread of toxins, anaerobic infections, in particular, may be fatal if not treated promptly. In addition, rapid development of edema, which constricts the muscle within its fascia, may lead to ischemic myonecrosis [12]. Sepsis: This is the most common complication of omphalitis. In a study by Mason and colleagues, bacteremia was a complication in 13% of infants with omphalitis. In these infants, disseminated intravascular coagulation (DIC) and multiple organ failure may occur [13]. Septic embolization: Cord infections delay or prevent obliteration of the umbilical vessels, so pathogens are thereby provided a direct access to the systemic circulation. If septic embolization arises from infected umbilical vessels, it may lead to metastatic foci in various organs, including the heart, liver, lungs, pancreas, kidneys, and skin [14]. 125 Abdominal complications: Abdominal complications include spontaneous evisceration, peritonitis, bowel obstruction, abdominal abscess, retroperitoneal abscess, or liver abscess. Long-term or late complications of omphalitis: Late complications occur several weeks, months, or years after omphalitis in the neonatal period. These may include nonneoplastic cavernous transformation of the portal vein, portal vein thrombosis, extrahepatic portal hypertension, and biliary obstruction. In one report of 200 patients undergoing portosystemic shunt for portal hypertension due to PVT, 15% of them were suspected to be the result of neonatal omphalitis. A portosystemic shunt may be required if portal hypertension develops [15]. Abscess formation of the falciform ligament in neonates is a known complication of omphalitis, where a soft tissue mass beneath the abdominal wall continuous with a thickened round ligament is a diagnostic feature of a falciform ligament abscess on USG or CT scanning. Many readily accessible abscesses are treated successfully with percutaneous drainage and antibiotics, but a successful treatment of the falciform ligament abscess is rather excision of the ligament itself (Fig. 26.9). Umbilical Hernia: Umbilical hernia is a common problem in children in Africa, and it may be a result of weakening of the umbilical cicatrix from neonatal omphalitis. This is discussed in Chap. 28. Umbilical granuloma may follow incompletely eradicated omphalitis due to chronic irritation, this will be discussed in Chap. 27. Peritoneal adhesions are the result of previous subclinical or treated peritonitis from omphalitis. The adhesions may produce intestinal obstruction, which usually is not amenable to nonoperative measures. Laparotomy and lysis/excision of the adhesions are usually required. 26.6 Treatment of Omphalitis Medical therapy: Include parenteral antimicrobial coverage for gram-positive and gramnegative organisms. A combination of an antistaphylococcal penicillin, vancomycin and an 126 aminoglycoside antibiotic is recommended. Some believe that anaerobic coverage is important in all patients [16]. Omphalitis complicated by necrotizing fasciitis or myonecrosis requires a more aggressive approach, with antimicrobial therapy directed at anaerobic organisms as well as gram-positive and gram-negative organisms. Metronidazole or clindamycin may provide anaerobic coverage [16]. Medical therapy is indicated only when infection is present. Antibiotics are also administered for acute infection of omphalomesenteric and urachal remnants. Supportive care is essential for survival, these measures include the following: • Infants should be treated at centers capable of supporting cardiopulmonary function. • Ventilatory assistance and supplementary oxygen for hypoxemia or apnea unresponsive to stimulation. • Intravenous fluid, vasoactive agents, or both (as indicated) for hypotension. • Administration of platelets, fresh frozen plasma, or cryoprecipitate for disseminated intravascular coagulation (DIC) if clinical bleeding is suggested. • In uncomplicated cases, erythema of the umbilical stump is expected to improve within 12–24 h after the initiation of antimicrobial therapy. Failure to respond may suggest disease progression, presence of an anatomic defect, or an immunodeficiency state. Surgical Care: Management of necrotizing fasciitis and myonecrosis involves early and complete surgical debridement of the affected tissue and muscle, with the following considerations: • Early surgical intervention may be lifesaving. • Delay in diagnosis or surgery allows progression and spread of necrosis, leading to extensive tissue loss and worsening systemic toxicity. • Although the extent of debridement depends on the viability of tissue and muscle, which is determined at the time of surgery, excision of preperitoneal tissue (including the umbilicus, umbilical vessels, and urachal remnant) is critically important in the eradication of the infection. 26 Omphalitis • These tissues can harbour an invasive bacteria and provide a route for progressive spread of infection after less extensive debridement. • Several surgical procedures may be required before all nonviable tissue is removed. • The mainstays of treatment for necrotizing omphalitis are early initiation of broadspectrum antibiotics, surgical debridement, large wounds may be sutured later or replaced with skin graft. • Intraperitoneal abscess or those located in the anterior abdominal wall and other locations should be drained at laparotomy, or accessed extraperitoneally if situated retroperitoneally. Omphalitis Prevention: Staphylococcal epidemics of pyoderma and omphalitis emerged, the umbilicus was found to be an important reservoir for dissemination of S. aureus. Prophylactic routine application of antimicrobial agents to the cord stump helped to control these epidemics. However, successes in preventing colonization by one organism sometimes resulted in colonization by others of equal or greater pathogenicity. The practice of applying, an antiseptic to the cord is now common not only in hospital nurseries but also outside hospitals, yet it has not been thoroughly evaluated. During the 1950s there were outbreaks of omphalitis that then led to anti-bacterial treatment of the umbilical cord stump as the new standard of care. It was later determined that in developed countries keeping the cord dry is sufficient, (known as ‘dry cord care’) as recommended by the American Academy of Pediatrics. The umbilical cord dries more quickly and separates more readily when exposed to air. However, each hospital/birthing center has its own recommendations for care of the umbilical cord after delivery. Some recommend not using any medicinal washes on the cord. Other popular recommendations include triple dye, betadine, bacitracin, or silver sulfadiazine, there is little data to support any one treatment (or lack thereof) over another. However one recent review of many studies supported the use of chlorhexidine treatment as a way to reduce risk of death by 23% and risk of omphalitis by anywhere between 27 and 56% in underdeveloped countries [16]. 26.7 26.7 Omphalitis in Adult 127 Omphalitis in Adult • Bacterial • Non bacterial Umbilical dermatitis is a common condition, it is usually associated with inadequate hygiene and deepening of umbilicus caused usually by obesity. The condition is really a dermatitis and analogous to intertrigo that often occurs between folds of the skin. Although it is primarily a ‘seborrhoeic’ dermatitis, but it frequently becomes secondarily infected with skin organisms. The whole umbilicus may feel hard with dermatitis, especially if the discharge is secondary to another condition such as an ompholith (or, very rarely, a tumour deposit). If the infection spreads into the subcutaneous tissues and the opening of the umbilicus becomes narrowed by oedema, the whole umbilicus can turn into an abscess. Cullen described umbilical concretions with local inflammation in the abdominal wall, and in reviewing many cases previously published as tuberculosis and infected dermoids, he considered most of them to be examples of primary umbilical sepsis [17]. Clinical presentation may be obvious with a red, hot, tender swelling with a Peau d’orange appearance in and around the umbilicus (Fig. 26.12). Bacterial omphalitis in adults may be in the form of cellulitis, and if a pus forming organisms (Staphylococci, streptococci, or rarely gram negative organisms) find a way to the deep umbilical tissues an abscess usually formed with exudation of pus (Figs. 26.13 and 26.14). Recurrent omphalitis at adulthood or older age should arouse the suspicious about the presence of congenital anomalies; like urachal or vitellointestinal remnants (Chaps. 35–36), or rare disorders like pilonidal sinus, omphalolithis or endometriosis in females (Chap. 31). Recently, omphalitis is a minor postoperative complication after laparoscopic procedures. It is treated quite simply as an outpatient problem, but omphalitis represents discomfort for the patient, and it can cause a delay in the resumption of work. Also, above all, omphalitis is a risk factor for the development of incisional Fig. 26.12 High power magnification of an early adult omphalitis Fig. 26.13 Pyogenic adult omphalitis, with a well formed abscess Fig. 26.14 Pus drainage from an umbilical abscess 128 umbilical hernia, which may occur in greater than 1% of cases [18]. Non bacterial adult omphalitis (Navel dermatitis): Specific and nonspecificic umbilical dermatitis may be a local manifestation of systemic dermatitis; like seborrhoeic dermatitis or psoriasis, but other chronic infection like fungus and tinea may which affect the umbilicus only, with its characteristic features, in many cases the primary focus of fungal infection start at the umbilicus and then spread to the abdominal wall, this commonly affect children and rarely may affect adults (Fig. 26.15), acute viral infection like herpes is not rarely to affect the umbilicus, specially in immunocompromised patients (Fig. 26.16). Primary irritant dermatitis: It is an exematous reaction of the skin caused by direct contact of toxic irritane substances. Fig. 26.15 Fungal omphalitis with spreading to the skin around the umbilicus Fig. 26.16 Herpes zoster omphalitis 26 Omphalitis 26.8 Navel Piercing Infection (Fig. 26.17) Navel piercing is one of the most common body piercings today. The Egyptian Pharaohs believed that earring at the navel is a sign of ritual transition from the life at the Earth to the eternity, it was popular among ancient Egyptian aristocrats, and was depicted in Egyptian statuary, also navel piercings are said to signify wealth and higher social status back in ancient society. But there is absolutely no proof either in sculptures, drawings, or even mummies that would point to the art of decorating the body with navel piercings during this time [19]. The actual navel is not pierced when a navel piercing is performed, the most common form of navel piercing is through the upper rim of the navel, rarely lateral umbilical rim is used and central piercing of the umbilical cicatrix is called the true navel piercing, which sometimes preferred by those who had a protruded umbilicus (outie). With the recent wide spread of navel piercing among girls, and sometimes men, by different types of jewellers, which not always done with aseptic techniques and by unqualified personals, there are many possible causes of navel piercing infection, with a wide spectrum of presentations; sometimes only cellulitis, which could be treated and controlled, but an umbilical abscess is not a rare sequel, (Fig. 26.18), other complications like kelloid formation will be discussed later (Sect. 31.6.2). Piercing omphalitis may be detected early after applying the jewellers if the pathogens find its way to the umbilical tissue from unsterile technique, or latter on due to bad hygiene, and improper care of the pierced navel. Having a tattoo near the pierced area; tight jeans for prolonged periods; carelessness in cleaning the navel; bathing in unclean waters; and frequent touching of the newly pierced navel are a predisposing factors for piercing omphalitis [20]. Umbilical piercing in particular can cause perioperative problems during laparoscopic procedures. 26.10 Rare Types of Omphalitis 129 Good skin hygiene and topical treatment as for intertrigo. Moderate/severe cases: as for mild cases, but if significant secondary infection detected then oral antibiotic is indicated to cover Staph aureus, once an abscess is suspected drainage is mandatory to avoid spread of infection to the underlying structures. Specific fungal or viral infection necessitate a specific lines of treatment. 26.10 Rare Types of Omphalitis Fig. 26.17 Navel piercing Fig. 26.18 Piercing omphalitis in a male 26.9 Management of Omphalitis in Adults Early and prompt treatment is mandatory to avoid serious complications; like liver abscess, and to reserve the normal athletic look of the navel. Mild cases: removal of foreign bodies such as hair-tufts or ompholiths. Myiasis omphalitis: Myiasis of the neonatal umbilicus is a rare disease with only a few reported cases in the literature, it is defined as the invasion of live mammalian tissue by the immature stage (maggots) of dipteran flies which feed on the host’s necrotic or living tissue. Although myiasis is mainly a disease of animals but humans may be affected, sometimes when they are reared in poor hygienic conditions [21]. Unhygienic practices coupled with traditional ways of handling newborn babies and the application of nonsterile instruments during and after delivery at the rural settings are the predisposing factors for myiasis omphalitis (Fig. 26.19). Medical care and access to maternity, health centres and hospitals will help to reduce home births and traditional handling of neonates. Cord care practices should be taught by qualified medical personnel to mothers and grandmothers who handle babies after birth. This should be practiced as a measure of prophylaxis to prevent the morbidity and mortality associated with myiasis as omphalitis infection persists [22]. Effective treatment of myiasis typically consists of the removal of the larvae, cleaning of the wound and use of local antiseptics and systemic antibiotics to control any possible associated infection. 26.10.1 Umbilical Tetanus Neonatorum (Fig. 26.20) Tetanus occurs worldwide and it was an important cause of neonatal deaths in developing coun- 130 Fig. 26.19 Myiasis omphalitis, with alive larvae retrieved from the umbilicus Fig. 26.20 Umbilical tetanus neonatorum, with spread of the infection to the penis and scrotum, a hands clenched to form a fist (claw hand) is noticeable tries. Tetanus infect an estimated 500,000 neonates each year with about 80% deaths in 12 tropical Asian-African countries alone. Till recently Neonatal tetanus accounted for 6.5% of deaths in infancy in India [23]. It is a rare serious infectious disease characterized by an acute onset of hypertonia, painful muscular contractions, and generalized muscle spasms. It is caused by contamination of umbilical stump with spores of clostrium tetani bacteria present in soil and faces of domestic animal and human, at 26 Omphalitis the time of cutting of cord, and use of unclean sharp weapon to cut the umbilical cord. In all deliveries by untrained person, cord was cut by unsterile instrument in many rural areas in developing countries. Application of ash on umbilical stump and improper maternal immunization during pregnancy are an important factors predisposing to tetanus omphalitis. Tetanic omphalitis is a fatal disease with a very high mortality rate, but it is a vaccine-preventable disease and it is considered as a failure of public health system [24]. Umbilical Tuberculosis: Abdominal tuberculosis also remains relevant in developing countries. It can be responsible for entero-umbilical fistula with purulent secretions and faeces, possibly in connection with a chronic under-umbilical inflammation in peritoneal tuberculosis. Lint ball omphalitis: ‘Belly-button lint ‘Foreign body-induced omphalitis’. Hairball is the most common type of foreign body seen in such cases. Most of the patients are young, hairy male with deep umbilicus with poor personal hygiene. A wide varieties of foreign body may be retrieved from the umbilicus and if it is retained for long time it may induce omphalitis, one interesting report of foreign body-induced umbilical discharge, is due to an old toilet paper ball in the umbilicus. Obesity, deep umbilicus, and poor hygiene may have been the predisposing factors for developing lint accumulation and subsequent omphalitis [25]. Omphalolith will be discussed with rare umbilical disorders in Sect. 31.3. Different types and lesions of umbilical dermatitis can be launched with photos in this site: http://medical-photographs.com/251-umbilical-lesions.html References 1. Bugaje MA, et al. “Omphalitis”. Paediatric surgery: a comprehensive text for Africa. Retrieved 23 July 2013. 2. 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