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Acta Pædiatrica, 2006; 95: 519 /522 CLINICAL OVERVIEW Neonatal omphalitis: A review of its serious complications NIA FRASER1, BRIAN W. DAVIES1 & JONATHAN CUSACK2 Departments of 1Paediatric Surgery and 2Neonatal Medicine, Queens Medical Centre, Nottingham, UK Abstract Until the advent of aseptic deliveries and aseptic umbilical cord care, many deaths occurred from umbilical infections. Omphalitis is a localized infection of the umbilical cord stump, most commonly caused by a single organism, which usually responds well to appropriate antibiotics. Umbilical sepsis is relatively uncommon in the developed world but is endemic in less-developed regions. Complications of omphalitis are exceedingly rare, but potentially catastrophic. Many of these babies will require surgical intervention. This paper aims to review the serious complications of omphalitis and how these should be managed. Conclusion: It is important to be aware of the rare but potentially lethal complications of omphalitis. Prompt recognition of serious sequelae is crucial for survival. Key Words: Necrotizing fasciitis, omphalitis, peritonitis, puourachus, umbilical sepsis, urachal remnant ‘‘The incidence of fatal sequelae in the cases that do occur is sufficiently great to merit serious consideration of the disease by obstetrician, paediatrician and surgeon.’’ J. W. Chamberlain, 1936 [1] The normal umbilical cord separates between 5 and 8 d after a vaginal delivery and about a day later following Caesarean section. Separation is probably initiated by thrombosis of the umbilical vessels, and contraction of certain proteins within their wall. Necrosis of the umbilical cord stump, by phagocytic neutrophils, occurs followed by epithelialization of the cord stump. The umbilicus may become colonized by bacteria from the maternal genital tract or from the environment soon after birth [2]. Cord infections delay or prevent obliteration of the umbilical vessels. Pathogens are thereby provided direct access to the systemic circulation. The estimated incidence of omphalitis in hospitalized newborns in the developed world is around 0.7% [3]. In developing countries, it may be as high as 6.18% [4]. There is a male preponderance. The most frequently encountered risk factors for the development of omphalitis in babies are protracted labour, non-sterile delivery, septic delivery as suggested by prolonged rupture of the fetal membranes or maternal infection, prematurity, low birthweight (B/2500 g), and umbilical catheters [4/6]. Some cases of omphalitis occur as a result of inappropriate cord handling, for example cultural application of substances such as tobacco ash [7]. Genetic defects in contractile proteins have also been implicated and, in some, immunological factors may play a part. Such abnormalities include defects in leukocyte adhesion molecules, neutrophil mobility and interferon production [8]. Omphalitis is characterized by periumbilical oedema, erythema, and tenderness with or without discharge [8]. Three categories are recognized based on the extent of infection [5]: 1) purulent discharge only; 2) cellulitis and lymphangitis of the abdominal wall; and 3) inflammation extending into the subcutaneous fat and deep fascia. The organisms responsible depend on geographical location. In the developed world, common pathogens include Staphylococcus aureus , Staphylococcus epidermi- Correspondence: N. Fraser, Department of Paediatric Surgery, Queens Medical Centre, Derby Road, Nottingham NG7 2UH, UK. Tel: /44 (0)115 924 9924. Fax: /44 (0)115 970 9929. E-mail: [email protected] (Received 19 January 2006; accepted 16 February 2006) ISSN 0803-5253 print/ISSN 1651-2227 online # 2006 Taylor & Francis DOI: 10.1080/08035250600640422 520 N. Fraser et al. dis , groups A and B Streptococcus, Escherichia coli , Klebsiella , Pseudomonas , and Clostridium difficile . Tetanus is an important pathogen in developing countries. Epidemics with organisms such as group A Streptococcus and Staphylococcus species may be implicated if there are clusters of babies presenting with omphalitis in nurseries [9]. In general, localized spread produces abdominal wall complications, while extension along umbilical or portal vessels results in intra-abdominal pathology. Babies in both groups are at risk of escalating systemic sepsis, and some may be moribund when first seen by a clinician. Serious complications of omphalitis that have been reported are listed in Table I. The typical clinical features of each and their management are reviewed. Septicaemia The signs of omphalitis-related neonatal infection are often very non-specific. Temperature instability, poor handling or abdominal distension should raise suspicion of sepsis. Systemic sepsis may lead to the seeding of septic emboli, causing problems such as abscesses, septic arthritis, meningitis [10] and bacterial endocarditis. Multiorgan failure and disseminated intravascular coagulopathy may result. The mortality rate for neonatal sepsis varies according to gestational age, but may be as high as 30% in very-low-birthweight (VLBW) babies [11]. Necrotizing fasciitis (NF) NF is a very rare, highly lethal, rapidly progressing soft tissue infection along an oedematous plane above the deep fascia. It is one of the most commonly reported major complications. Necrosis may spread to involve skin overlying the chest wall, flanks and scrotum, and typically mixed aerobes and anaerobes are isolated [12,13]. The baby initially appears deceptively well [7], but systemic toxicity soon escalates leading to a mortality rate of between 50 and 87.5% [6,14]. Oedema of the umbilicus and peau d’orange appearance, which implies obstruction of the deep Table I. Major complications of neonatal omphalitis. Complication Septicaemia Necrotizing fasciitis Abscesses: Retroperitoneal, pelvic, cutaneous, hepatic Peritonitis Adhesive small bowel obstruction Spontaneous small bowel evisceration Hepatic vein thrombosis lymphatics, is a warning sign of a salvageable situation. If unrecognized, the skin very quickly forms a purplish-blue discolouration pathognomonic of NF, then sloughs. Very rarely, gangrene may extend through the rectus abdominis and peritoneum, and even involve the adjacent bowel [7]. It is important to note that crepitus is not a typical feature of umbilical NF. Survival depends on prompt recognition of early signs, and aggressive resuscitation followed by extensive surgical debridement. This procedure involves excision of all necrotic material, until healthy bleeding tissue is reached [15]. The importance of the excision of umbilical vessels has been emphasized by Dinari [16] and Kosloske [13], even if they appear macroscopically intact [14]. Multiple debridements may be necessary [17], and the resulting wounds are left open. Drains and skin grafts are sometimes required. The rapid progression of untreated disease is aided by bacterial release of collagenase and toxins, and polymicrobial infections carry the worst prognosis. Some authors have suggested that the use of hyperbaric oxygen may minimize disease progression [14]. Immunoglobulin has also been used as a treatment modality by some [15]. Peritoneal complications In the neonate, the umbilical vein is separated from the peritoneal cavity by only a thin layer of areolar tissue and a single layer of peritoneum. This makes the abdominal cavity vulnerable in the face of umbilical sepsis. If the umbilical vein becomes infected, peritoneal contamination often becomes generalized because the neonatal omentum is small and cannot effectively wall off infection. Babies with omphalitis complicated by peritonitis are unable to feed and may have bilious vomiting. Diarrhoea is present in 50% of cases. The neonate appears septic, and the abdomen is distended and tender. Laparotomy is usually performed if other pathologies such as malrotation and volvulus cannot be excluded. During the procedure, all intraperitoneal pus is evacuated and involved umbilical vessels excised. Interestingly, Holve [18], Duggen [19] and Bell [20] described several babies with this condition who were managed conservatively with parenteral antibiotics. The authors stress that a ‘‘surgical’’ abdomen must first be excluded. Chadwick successfully treated one baby with intraperitoneal followed by intravenous antibiotics [21]. Adhesive small bowel obstruction was reported as a late complication by Ameh in a 75-dold boy with previous neonatal omphalitis [22]. At laparotomy, there were multiple adhesions between loops of the small bowel and the anterior abdominal wall in the region of the umbilicus. Neonatal omphalitis Infection of urachal remnant The fetal urachus develops from the apex of the ventral cloaca and is a tubular structure connecting the bladder to the umbilicus. Normally, as the bladder descends behind the pelvic brim, the urachus elongates then undergoes luminal obliteration. Incomplete obliteration may lead to the formation of urachal cysts or communication with the umbilicus or bladder. Babies with a urachus communicating with an infected umbilicus may present with unresolving omphalitis and, in extreme cases, pus will fill this structure and form a urachal abscess (pyourachus). Macmillan reported two babies with perforation of pyourachus and resultant peritonitis, both of whom survived following abscess drainage and excision of urachal remnants [23]. 521 utilizing an abdominal incision if the collection is more anterior. The cavity is lavaged and drains may be beneficial. Hepatic complications The hepatic portal vein may become obstructed due to ascending infection and thrombosis (portal cavernoma). Varices may develop and require sclerotherapy, and portal-systemic shunts may be necessary [25]. Secondary biliary tract obstruction has also been reported [26]. Hepatic abscesses may occur, and spontaneous rupture and necrosis of the entire left lobe has been described [22]. These abscesses are managed by extraperitoneal drainage or laparotomy. Spontaneous bowel evisceration Abscesses Superficial cutaneous abscesses may be confined to the umbilical region or occur in distant sites due to haematogenous seeding of septic emboli. Intrabdominal abscesses may arise if bacteria originating in the umbilicus invade the umbilical artery. This vessel joins the iliac artery in the pelvis, and infection may extend along these structures to form retroperitoneal or pelvic abscesses (Figure 1). Depending on the exact location, the baby may present with a lower abdominal mass or inguinoscrotal swelling [16]. Feo reported a neonate with a retroperitoneal abscess, which extended into the gluteal region [24]. Following diagnosis, surgical drainage is best performed at the site where the abscess points, or by Umbilical vein Umbilical cicatrix Umbilical artery Urachus Bladder Figure 1. Anatomy of the anterior abdominal wall as seen from inside the peritoneal cavity This is extremely rare and occurs as a result of the breakdown of fascia forming the umbilical cicatrix and overlying skin. The eviscerated bowel may be strangulated, resulting in gangrene [22]. Necrotic bowel should be resected, and the remainder cleaned and returned to the peritoneal cavity. The umbilicus is then reconstructed. Summary Omphalitis is a potentially lethal infection and should never be underestimated. Neonates, in particular those born prematurely, have not yet reached immunological maturity [20]. For this reason, in part, a minor umbilical infection may rapidly lead to a serious complication. The umbilical vein provides a direct route for bacteria to the liver, and the umbilical arteries and urachus are pathways to the pelvis. In order to prevent complications associated with omphalitis, a baby with periumbilical cellulitis should be admitted for intravenous antibiotics effective against Staphylococcus aureus , streptococci and Gram-negative rods. If infection persists despite appropriate treatment, an underlying infective focus such as a patent urachus should be sought. The principles of the management of complicated omphalitis include ventilatory support and fluid resuscitation to correct dehydration, acidosis and associated problems. A Gram stain and culture of umbilical exudate or necrotic material should be performed prior to the administration of systemic antibiotics. The early recognition of umbilical oedema and peau d’orange is vital for the baby’s survival in cases of NF. This sign, or the blue discolouration pathognmonic of NF, should prompt surgical referral for extensive debridement. Hyperbaric oxygen and immunoglobulin could be considered if available. 522 N. Fraser et al. Suspected peritonitis usually warrants laparotomy, and abscesses and urachal remnants should be drained or excised as appropriate. Cord infections in developing countries can be prevented through increasing access to tetanus toxoid immunization during pregnancy, promoting clean cord care and reducing harmful cord applications. Garner et al. reported a successful intervention programme in Papua New Guinea, which included the provision of both health education and umbilical cord care packs. This led to a decline in deaths from neonatal omphalitis [27]. A recent Cochrane systematic review was unable to make recommendations for best practice regarding cord care, but suggested that the use of topical antiseptics in higher-risk preterm infants may be beneficial [28]. Vigilance in identifying the major complications of neonatal omphalitis and early referral to specialist centres is vital for the survival of these babies. [11] Philip AGS. The changing face of neonatal infection: experience at a regional medical centre. Pediatr Infect Dis 1994;13: 1098 /102. [12] Kosloske AM, Bartow SA. Debridement of periumbilical necrotizing fasciitis: importance of excision of the umbilical vessels and urachal remnant. J Pediatr Surg 1991;26:808 /10. 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