Pediatr Clin N Am 51 (2004) 819 – 827

Anomalies, abnormalities, and care of

the umbilicus
Albert Pomeranz, MD
Department of Pediatrics, Medical College of Wisconsin, Children’s Hospital of Wisconsin,
Downtown Health Center, 1020 North 12th Street, Milwaukee, WI 53233, USA

The umbilical cord, along with the anterior abdominal wall, begins develop-
ment by the end of the third week of gestation. By the twelfth week, embryonic
remnants leave one umbilical vein and two umbilical arteries covered by amnion,
which are eventually protected and incased by Wharton’s jelly. Although it is
shed after birth, remnants remain clinically relevant in the newborn period and
early childhood. This article discusses abnormalities that involve the umbilical
cord and issues related to its care after birth.

Umbilical cord anomalies and abnormalities

Cord characteristics
The mean length of the cord is 55 cm [1]. Short cords are associated with
decreased fetal movement as a result of either maternal or fetal causes. Maternal
causes include oligohydramnios and uterine abnormalities that result in less fetal
space; fetal causes include structural limb defects, such as amniotic bands and
neurologic limb defects, as seen in Werdnig-Hoffman syndrome [2]. Long cords
( > 70 cm) are associated with fetal entanglements, true knots, and cord prolapse.
The umbilical cord contains several loops or coils that are established early in
fetal development. These coils are believed to supply protection from compressive
forces placed on the cord. Abnormal coiling of the cord is associated with various
adverse neonatal outcomes, including premature delivery and fetal demise [3– 5].

Single umbilical artery

A single umbilical artery (SUA) is a relatively common congenital malfor-
mation and often is associated with other significant malformations. In a meta-

E-mail address: pomeranz@mcw.edu

0031-3955/04/$ – see front matter D 2004 Elsevier Inc. All rights reserved.
doi:10.1016/j.pcl.2004.01.010
820 A. Pomeranz / Pediatr Clin N Am 51 (2004) 819–827

analysis in which the diagnosis was made by examining placental and umbilical
cord specimens from live born infants or by examining the umbilicus after birth,
the mean incidence was 0.55%, with 27% of these having an associated mal-
formation [6]. This statistic emphasizes the importance of examining the new-
born cord closely and performing a careful search for additional abnormalities
if a two-vessel cord is detected. The associated malformations varied, with a
substantial number being multiple and having associated chromosomal abnor-
malities. Although the increased risk of morbidity and mortality in newborns
with SUA is largely related to the concurrent fetal and placental malforma-
tions, even nonmalformed infants with SUA may have an increased perinatal
mortality rate [4].
The controversial subject is what to do about isolated SUA without any
physical evidence of associated anomalies. In the meta-analysis of the newborns
with isolated SUA in which urologic evaluations were performed, 16% had renal
anomalies [6]; however, 54% of these were considered minor or self-limiting,
which left 7.3% of cases as clinically significant. The authors of the study believed
that screening was not cost effective and that many of the anomalies would be
diagnosed in follow-up, with the value of early detection unclear. The number
of patients (204) in this study with asymptomatic SUA was relatively small, and
no control groups without SUA were available.
The results and opinion of the authors of another study illustrated the sub-
jectivity involved in the recommendation regarding screening of newborns with
isolated SUA. In the study by Bourke et al [7], which was one of the studies
included in the previously discussed meta-analysis, the authors performed screen-
ing ultrasounds over a 6-year period on all newborns born in their hospital with
isolated SUA. They identified 112 cases and found significant renal abnormalities
in 8 (7.1%) of these newborns. Four of these patients had grade 2-3 ureteropelvic
reflux, yet the authors believed that screening was justified. Until a prospec-
tive study that compares the rate of significant renal abnormalities in isolated
SUA with a healthy control group is performed, a firm recommendation cannot
be made. Currently, however, this author is in favor of screening these infants
with renal ultrasound.

Urachal abnormalities
The urachus is an embryonic remnant of the communication between the
bladder and the umbilicus and generally exists as a fibrous cord. Four clinical
anomalies have been described: urachal sinus, urachal cyst, patent urachus, and
urachal diverticulum [8]. In a study of 48 patients by Cilento et al [8], the pre-
senting complaint was periumbilical discharge in 42%, followed by a mass or
cyst and abdominal or periumblical pain. In another study of 21 patients, 57%
presented with periumbilical discharge [9]. The most common anomalies were
urachal sinus and urachal cyst.
The urachal sinus may present with drainage, pain, or periumbilical redness
[9]. If the discharge empties into the bladder, symptoms of a urinary tract in-
 A. Pomeranz / Pediatr Clin N Am 51 (2004) 819–827 821

fection may occur [8]. The differential diagnosis includes a patent urachus,
patent omphalomesenteric duct, umbilical granuloma, and omphalitis. If an
opening can be cannulated, sinography is the study of choice because it allows
the diagnosis of a patent urachus or patent omphalomesenteric duct that
communicates with the bowel [8].
Urachal cysts present in several ways, typically in early childhood. Although
urachal cysts may present as a palpable periumbilical mass, they most often pre-
sent as an infected cyst with abdominal pain, erythema, and periumbilical swel-
ling, generally below the umbilicus. The cyst may rupture and drain from the
umbilicus or intraperitoneally, which results in an acute abdomen. Staphylococ-
cus aureus is the most common isolated organism. Occasionally they may be
detected incidentally during other radiologic procedures secondary to calcifica-
tion. The diagnosis usually can be made by ultrasound [8,9].
A patent urachus involves free communication between the bladder and the
umbilicus, which allows urine to flow from the umbilicus. It presents with a per-
sistent wet or draining cord and occasionally as a urinary tract infection [10]. The
drainage is usually thin and clear. The differential diagnosis includes abnormali-
ties that result in a wet or draining cord, similar to those discussed for a urachal
sinus. The umbilical granuloma may be distinguished by the nature of the dis-
charge and presence of the granuloma on examination. A sinogram aids in
diagnosis in most cases and should reveal the presence of a patent omphalome-
senteric duct. A cystogram is often still recommended to rule out a urinary
obstructive lesion, such as posterior urethral valves. The patent urachus is be-
lieved to act as a ‘‘pop off’’ valve in these obstructed cases [11].

Omphalomesenteric duct (vitelline) abnormalities

The omphalomesenteric duct forms a connection between the intestinal tract
and abdominal wall during fetal development. Several possible remnants are pro-
duced, the most common being a Meckel’s diverticulum. Symptomatic Meckel’s
diverticulum most commonly presents in the young child as painless rectal bleed-
ing from ectopic gastric mucosa, although it may be the lead point for intussus-
ception in neonates and infants. If the duct remains patent to the umbilicus, fecal
drainage from the umbilicus can occur. The differential diagnosis includes the
urachal remnants and an umbilical granuloma, but the nature of the discharge
is often helpful [10,11]. Other remnants include bands, which result from the
attachment between the abdominal wall and intestine, and cysts. These bands
may result in volvulus and intestinal obstruction; cysts may become infected and
less commonly result in obstruction.
In a large study of 217 pediatric cases of omphalomesenteric duct remnants,
neonates and infants most commonly presented with intestinal obstruction [12].
Three neonates and one older infant (6 weeks old) with a patent omphalomesen-
teric duct all presented with bilious drainage from the umbilicus. An umbilical
sinogram with contrast aided in diagnosis in these cases. All of the urachal and
omphalomesenteric abnormalities require surgical intervention.
822 A. Pomeranz / Pediatr Clin N Am 51 (2004) 819–827

Umbilical granuloma
Umbilical granulomas commonly come to the attention of parents because
of persistent drainage or moisture involving the umbilicus after the cord has
dried and separated. It is not a congenital abnormality but represents continuing
inflammation of granulation tissue that has not yet epithelialized. The lesion is a
round, wet, often pink, pedunculated lesion of variable size, usually 3 to 10 mm.
The contribution of colonized bacteria and low-grade infection is unclear. Ura-
chal duct and omphalomesenteric duct remnants that produce umbilical wet-
ness or discharge must be considered in the differential diagnosis. Umbilical
polyps, which represent a distal omphalomesenteric remnant, appear similar to a
granuloma. Failure of a presumed umbilical granuloma to respond to treatment
with silver nitrate should raise suspicion that one is dealing with one of these
other abnormalities.
The most commonly used treatment of umbilical granuloma is silver nitrate
cauterization that comes mounted on a wooden stick applicator in a concentration
of 75%. This chemical acts as an antiseptic, astringent, or caustic agent depend-
ing on the concentration [13]. The application may be repeated every few days
as necessary but generally requires only a few treatments for success. Its use
can result in burns to periumbilical skin, so caution should be exercised during
application [14]. Surgical removal of the granuloma rarely is required.

Umbilical cord care, omphalitis, and necrotizing fasciitis

Umbilical cord care

The umbilicus is often the first site of colonization of S aureus and other
organisms [15]. The presence of S aureus and the degree of colonization increase
the risk of infection at the periumbilical site and other sites [16,17]. The care
of the umbilicus in the newborn period has been directed at controlling this
colonization in hopes of decreasing infection, particularly omphalitis and
fasciitis. Various antibiotic and antiseptic agents have been used for this pur-
pose and have been demonstrated to reduce colonization and infection rates
[18 –21]. Group B streptococcus colonization rates also have been inhibited
by various antimicrobial agents [19,22].
Several different antimicrobial agents have been used to reduce colonization
rates, including triple dye, bacitracin, silver sulfadiazine, povidone-iodine, chlor-
hexidine, and hexachlorophene. Although a recent Cochrane Review article
was unable to determine the best cord care in developed countries, all of the
agents mentioned previously have been shown to reduce colonization rates, but
their effectiveness varies by the study [23]. Alcohol is not effective in controlling
colonization, nor does it seem to shorten the time until cord separation [24].
Triple dye is one of the more commonly used antibacterial agents, and it
seems to be effective at reducing group B streptococcus colonization and S aureus
 A. Pomeranz / Pediatr Clin N Am 51 (2004) 819–827 823

[20,22,25,26]. More than other agents, however, this agent results in prolonged
cord separation times [27] and leaves the cord discolored. Side affects or toxici-
ties from the other agents rarely have been reported. Hexachlorophene bathing
of infants was common up to 1971, at which time the Food and Drug Adminis-
tration and American Academy of Pediatrics issued a statement discouraging its
use because of fear concerning possible neurotoxicity after cutaneous absorption
[28]. Its use on a small surface area, such as the umbilical stump, is not viewed as
a problem, however. When used extensively and repeatedly on the newborn,
povidone-iodine has been reported to result in transient thyroid suppression
[29], and there is some concern over the possibility of falsely elevated thyroid-
stimulating hormone levels on the newborn screen when used only on the cord.
A study conducted by Lin et al [30] demonstrated significantly more false-positive
thyroid-stimulating hormone levels in screened newborns on whose umbilical
cords povidone-iodine was used compared with alcohol or triple dye.
Several hospitals have discontinued antimicrobial treatment of the cord, which
is partially a result of changes in maternity care. S aureus cross-contamination
of the umbilical stump from infant to infant via hospital personnel has been
demonstrated [31]. It is assumed that the increase in single rooms and infants
‘‘rooming-in’’ with mothers should result in fewer personnel being involved and,
therefore, less colonization [20]. Significant omphalitis and its major complica-
tion, necrotizing fasciitis, also are relatively rare [32,33].
A recent study by Janssen et al [20] illustrated the uncertainty surrounding
cord care. The authors reported on a randomized trial that involved 766 newborns
on whom triple dye/alcohol versus dry cord care was used; the dry cord care
consisted of soap and water to the periumbilical area. Although only one case of
omphalitis was reported in the dry cord care group compared with the treated
group, which was not statistically significant, there was significantly greater
colonization of S aureus, group B streptococcus, and Escherichia coli. This
statistic is concerning because of the association of S aureus colonization,
particularly when heavy with subsequent infection [34]. Community nurses also
observed cord exudates and foul odor significantly more often in the dry cord
care group during the home visit.
Prolonged cord separation times are a concern to parents and physicians.
Antimicrobial treatment of the cord is considered to be a major cause [27,35].
A suggested explanation for this effect is that the lower colonization rates result
in a decrease in leukocyte infiltration of the cord and its subsequent digestion
by leukocytes. The mean cord separation times are reported to be 7 days with
dry cord care [36] and approximately 15 days with triple dye [37,38]. In a study
by Wilson et al [38], 10% of cords to which triple dye was applied in the nur-
sery were still attached at 3 weeks.
Prolonged cord separation is important primarily for two reasons. The delay in
separation may result in anxiety on the part of parents and health care personnel
[39]. The delayed separation, particularly when associated with omphalitis, also
may be the first sign of leukocyte adhesion deficiency. This is a rare disorder
of neutrophils characterized by defective adhesion-dependent functions, such
824 A. Pomeranz / Pediatr Clin N Am 51 (2004) 819–827

as chemotaxis, which result in severe recurrent infections during infancy [40,41].

In most cases, cord separation was delayed beyond 3 weeks, with some infections
presenting after cord separation. Newborns with cord separation times beyond
3 weeks should be monitored for signs of omphalitis and other infections
commonly seen with this disease, such as aseptic meningitis, perianal infections,
and otitis media [41]. Because these infants often have persistent leukocytosis
with significant neutrophilia even when not infected [41], it is reasonable to
screen asymptomatic infants with delayed cord separation times, such as beyond
4 weeks, with a complete blood count with differential. If leukocytosis is present,
further immunologic evaluation is indicated. A less serious form of the disease,
leukocyte adhesion deficiency type II, results from partial absence of the ad-
hesion molecule.
In addition to the previously discussed causes of delayed separation of the
cord, Razvi et al [42] reported two infants with delayed cord separation attrib-
uted to urachal cysts. Both infants were diagnosed by ultrasound and had negative
test results for immune defects. One infant had developed umbilical discharge
at 8 weeks of age.
In conclusion, when dry cord care of the umbilicus is used, parents must be
educated about signs of infection and the need to contact their health care pro-
vider immediately if any of these signs are present. In less developed countries,
where adequate cord care cannot be guaranteed, application of antimicrobial
and aseptic agents to the cord is recommended.

Omphalitis
Omphalitis is an infection of the umbilical cord stump or the surrounding
tissue that results from organisms that have colonized the area. One of the main
reasons for antiseptic treatment of the umbilical cord in the newborn is preven-
tion of this disease. Because the umbilicus presents access to the portal vein via
the umbilical vein, infection in this area may result in portal vein thrombosis,
phlebitis, and liver abscesses. A long-term complication is extrahepatic portal
hypertension. The process also may spread through fascial plains and result in
peritonitis [43]. Although rare, the development of necrotizing fasciitis may
occur and is associated with a high mortality rate [33,44]. Omphalitis is a
polymicrobial infection; the most common organisms involved include S aureus,
group A streptococcus, and gram-negative bacteria [44]. Although anaerobes
rarely have been documented in this infection, they have been recovered in up
to 39% of patients when looked for aggressively [44,45].
The clinical presentation of omphalitis is characterized by drainage from the
umbilical stump and signs of periumbilical inflammation, such as erythema
induration and swelling of the skin. Associated systemic signs, including leth-
argy, fever, irritability, and poor feeding, indicate more severe infection or
complications. The difficulty with the diagnosis lies with the mild presentation
of discharge from the umbilical stump without inflammatory signs, particularly
after cord separation. This occurrence may be normal even when associated
 A. Pomeranz / Pediatr Clin N Am 51 (2004) 819–827 825

with some odor [44]. Some physicians treat infants with minimal symptoms with
a topical agent, such as alcohol, and application of topical antibiotics, such as
bacitracin or mupirocin [44], although there is no evidence of the efficacy of
this practice. Infants with omphalitis must be followed closely for any signs
of inflammation, particularly erythema of the periumbilical skin beyond a short
distance (>0.5 cm) from contact with the cord [20]. In infants without inflamma-
tion, it is important to consider other causes of moisture or discharge from
the cord, including umbilical granuloma and urachal and omphalomesenteric
duct anomalies.
Patients with omphalitis who have signs of inflammation should have the
discharge or pus cultured after the skin has been decontaminated and blood
cultures obtained, particularly if they are febrile. Investigation of other sites where
infection may have spread is indicated based on the clinical status of the patient.
Systemic antibiotics that cover the organisms most commonly involved, including
S aureus, streptococcus, and gram-negative bacteria, are necessary. Coverage
for anaerobes also should be considered, especially for infants with foul-smelling
drainage or infants born of mothers with amnionitis [44]. In cases that involve
mild signs of inflammation but no systemic signs or symptoms, oral antibiotic
therapy may be reasonable but must be accompanied by close follow-up [43].

Necrotizing fasciitis
Necrotizing fasciitis is a rare, often fatal complication of omphalitis that
involves infection of the skin, subcutaneous fat, and surperficial and deep fascia.
It progresses rapidly. In a review of the literature from 1999 [32], which yielded
66 cases of necrotizing fasciitis, the overall mortality rate was 59%. Most cases
were secondary to omphalitis and were polymicrobial. The most common aerobic
bacteria were S aureus, E coli, and enterococcus; clostridium species and bac-
teroides were the predominant anaerobic bacteria. The rapid progression of in-
flammation along with marked tissue edema and signs of systemic toxicity are
characteristic of the infection [44]. Early diagnosis is essential because in
addition to antibiotic therapy, immediate surgical débridement results in the
greatest chance for survival.

Summary
An SUA is the most common anomaly of the umbilical cord and is often
associated with other congenital anomalies. When present as an isolated phenom-
enon without other physical abnormalities, the decision to screen for associated
renal anomalies is debatable but recommended by this author. Urachal and
omphalomesenteric anomalies may present in the newborn period with umbilical
cord drainage or wetness. They must be distinguished from an umbilical granu-
loma, which is by far the more common abnormality.
826 A. Pomeranz / Pediatr Clin N Am 51 (2004) 819–827

The use of antimicrobial and antiseptics agents on the umbilical stump pre-
vents bacterial colonization and infection, particularly omphalitis and necrotizing
fasciitis. With changes in maternal care resulting in less bacterial cross-contami-
nation of infants and the relative rarity of these infections, many hospitals have
adopted dry cord care. In this situation, parents must be educated carefully about
signs of infection and the need to contact a health care provider immediately if
present. Although long cord separation times may result from the use of anti-
microbial agents on the cord stump, they also may be associated with leukocyte
adhesion abnormalities, especially when associated with infections in the neonatal
period. For infants whose cords persist beyond 3 to 4 weeks without associated
infection, a screening complete blood count to detect leukocytosis is a reasonable
screen for this disorder. If leukocytosis is present or neonatal infection occurs,
further immunologic testing is required.

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