Umbilical cord prolapse

INTRODUCTION Umbilical cord prolapse is a rare obstetrical emergency that

occurs when the umbilical cord descends alongside or beyond the fetal presenting part. It is lifethreatening to the fetus since blood flow through the umbilical vessels is usually compromised
from compression of the cord between the fetus and the uterus, cervix, or pelvic inlet. There are
two types of cord prolapse:

Overt prolapse, which is the most common, refers to protrusion of the cord in advance of
the fetal presenting part, often through the cervical os and into or beyond the vagina. The
fetal membranes are invariably ruptured in these cases and the cord is visible or palpable
on examination.

Occult prolapse occurs when the cord descends alongside, but not past, the presenting
part. It can occur with intact or ruptured membranes. The diagnosis should be considered
in the differential diagnosis of a sudden, prolonged fetal heart rate deceleration. An occult
prolapse often cannot be diagnosed with certainty, but is suggested by clinical features
(eg, fetal bradycardia) and findings at cesarean delivery.

INCIDENCE Cord prolapse occurs in 0.14 to 0.62 percent of deliveries [ 1 ].

RISK FACTORS AND ETIOLOGY Pregnancy characteristics that increase the risk of cord
prolapse are generally not modifiable, but awareness of patients at high risk may help facilitate
prompt diagnosis and delivery when prolapse occurs. The two major etiologic categories of cord
prolapse are:

Fetomaternal factors that lead to inadequate filling of the maternal pelvis by the fetus

Iatrogenic obstetrical interventions

Fetomaternal factors Risk factors associated with cord prolapse that are nonmodifiable are
listed in the table ( table 1 ). Selected risk factors are briefly discussed below:

Fetal malpresentation Nonvertex fetal presentation is consistently associated with a

high risk of cord prolapse [ 1-4 ]. In one review, the overall frequency of cord prolapse in
vertex, breech, and transverse lies was 0.24, 3.5, and 9.6 percent, respectively [ 2 ].
Footling breech presentation carries a higher risk of cord prolapse than other types of
breech presentation. Nevertheless, most cord prolapses occur with vertex presentations
because of the relatively low incidence of noncephalic presentation.

Prematurity Infants delivering prematurely have a higher rate of cord prolapse,

probably due to the smaller size of the fetus relative to the amniotic fluid volume and the
increased frequency of malpresentation among premature fetuses [ 1,3,5 ].

Multiple gestation The risk of cord prolapse in a term twin pregnancy is confined to
the second born twin, in whom there is an increased probability of malpresentation [ 1,57 ].

Multiparity Cord prolapse in multiparous women may be related to the increased

likelihood of rupture of membranes prior to engagement of the presenting part, since
engagement in multiparas often occurs after labor has begun and later than in nulliparas [
4 ]. A study of 30 patients with cord prolapse reported that two-thirds of the women were
para six or more; one patient experienced cord prolapse in two successive deliveries [ 8 ].

Rupture of membranes Iatrogenic or spontaneous rupture of membranes can lead to

cord prolapse because a forceful gush of fluid may carry the cord beyond the presenting
fetal part. The highest risk of this occurrence is with preterm premature rupture of
membranes, polyhydramnios, or an unengaged fetal presenting part.

Polyhydramnios Polyhydramnios is often associated with an unstable lie or

unengaged presenting part, as well as copious flow of amniotic fluid after membrane
rupture [ 9 ]. As discussed above, all of these factors increase the risk of cord prolapse.

Iatrogenic obstetrical intervention The evidence that iatrogenic obstetrical intervention

causes cord prolapse is conflicting and it is often difficult to determine whether cord prolapse
would have occurred spontaneously if the intervention had not been performed [ 10 ]. The
purported mechanism for cord prolapse in this setting is that disengagement of the fetal head
during the intervention and a high outward flow of amniotic fluid allow the umbilical cord to be
carried into the birth canal.
Interventions that may predispose to cord prolapse include:

Iatrogenic rupture of membranes

Application of an internal scalp electrode

Insertion of an intrauterine pressure catheter

Manual rotation of the fetal head

Amnioinfusion or amnioreduction

External cephalic version in patients with ruptured membranes [ 11 ]

Application of forceps or vacuum

Some of these interventions are more commonly performed during labor induction; therefore, not
unexpectedly, cord prolapse has been reported to occur more commonly with induced than
spontaneous labor [ 12 ]. (See 'Prevention' below.)

CLINICAL MANIFESTATIONS AND DIAGNOSIS The first sign of cord prolapse is

usually a severe, prolonged fetal bradycardia or moderate to severe variable decelerations after a
previously normal tracing [ 1,13 ]. Cord prolapse should always be suspected where the
abnormal fetal heart rate pattern develops soon after membrane rupture.
The cord is palpable upon vaginal examination if there is overt prolapse, but it may not be
readily identified if the prolapse is occult, particularly when membranes are intact. Less
commonly, the patient may see or feel an overt cord prolapse or the care provider may initially
palpate a pulsating cord on a vaginal examination performed to assess labor progress.
If cord prolapse is not overt, the suspected diagnosis cannot be confirmed until a cesarean
delivery is performed, and may not be apparent even at surgery.
In one large series, mean cervical dilatation at the time of cord prolapse was 5.8 cm, but prolapse
can occur at any cervical dilation from minimal to full dilatation [ 10 ]. The mean station in this
series was -1.6, which is expected since an engaged presenting part generally prevents the cord
from prolapsing.
Differential diagnosis Other causes of fetal bradycardia of abrupt onset following a normal
tracing include maternal hypotension, placental abruption, uterine rupture, and vasa previa. Fetal
bradycardia following injection of an epidural anesthetic suggests maternal hypotension from
vasodilatation; this usually responds to intravenous fluid infusion and ephedrine. In contrast to
cord prolapse, placental abruption, uterine rupture, and vasa previa are usually accompanied by
vaginal bleeding, and abruption and rupture are often painful in the absence of labor anesthesia.
MANAGEMENT The optimal obstetrical management of cord prolapse is prompt delivery to
avoid fetal compromise or death from compression of the cord between the presenting fetal part
and the margin of the pelvic inlet. Although immediate cesarean birth is generally the optimal
mode of delivery, vaginal delivery may be considered in select situations
There are no data from prospective or randomized studies on which to base management
recommendations because of the infrequent and urgent nature of this problem. There are sparse
data regarding attempted vaginal delivery in the setting of cord prolapse. Successful operative
vaginal delivery with vacuum or forceps has been reported when cesarean delivery could not be
performed immediately. One series of 93 cases of cord prolapse from a single center noted
successful vaginal delivery in one third [ 14 ]. Vaginal delivery was most likely in patients at full
dilation with breech presentation or undergoing birth of the second twin at the time of the event.
The event to delivery time interval was significantly faster in patients who underwent cesarean
delivery.
Intrauterine resuscitation The following preoperative maneuvers can be helpful for reducing
pressure on the cord. In the absence of comparative studies, there is no evidence that one
maneuver is better than another.
Manipulation of the cord and exposure to a cool environment may exacerbate poor perfusion by
inducing spasm of the cord vessels, so these factors should be minimized.

Funic decompression This is the most common method of alleviating cord compression, and
the maneuver we prefer. After diagnosis of cord prolapse, the examiner's hand is placed in the
vagina and used to elevate the fetal head off of the cord while preparations for an emergency
cesarean delivery are made. The patient can be placed in steep Trendelenburg or the knee-chest
position to try to move the fetus and further alleviate cord occlusion.
Bladder filling Another temporizing technique involves placing the patient in Trendelenburg
position and inserting a Foley catheter into the bladder; the bladder is then rapidly filled with 500
to 700 milliliters of normal saline. The distended bladder elevates the presenting part and keeps it
off of the cord, thus relieving the compression and potentially obviating the need for prolonged
vaginal digital decompression. Bladder filling may be particularly useful when immediate
cesarean delivery is not possible.
One series of 51 cases employed both a tocolytic (ritodrine) and bladder filling to relieve cord
compression [ 15 ]. The mean interval from diagnosis to delivery was 35 minutes and there were
no fetal or neonatal deaths; the majority of infants had Apgar scores 7 at five minutes.
Funic reduction Funic reduction is a controversial approach to the management of cord
prolapse. As with decompression, it is employed to alleviate pressure on the cord from the
presenting part while preparations for cesarean delivery are being made. The procedure involves
elevating the fetal head with gentle suprapubic pressure or transvaginally or both and then sliding
the cord over the widest part of the vertex and placing it in the nuchal area. Gentle suprapubic
fixation of the head decreases the chance of creating an oblique or transverse lie.
Funic reduction had been considered an appropriate initial step in management of cord prolapse,
but was subsequently discarded in favor of cesarean delivery because the procedure appeared to
be associated with an increase in intrapartum asphyxia and demise [ 16 ]. However, this
association was observed prior to the advent of continuous fetal monitoring. With use of
continuous fetal monitoring, this technique may permit vaginal delivery and avoid emergent
cesarean delivery in some patients. This was suggested by a small series of eight patients with
cord prolapse in which five vaginal deliveries were achieved 14 to 512 minutes after a successful
funic reduction without anesthesia [ 16 ]. The other three patients either delivered vaginally
shortly after the diagnosis of cord prolapse or were delivered by cesarean. All of the fetuses were
in vertex presentation and had less than 25 cm of cord prolapsing. No morbidity or mortality was
reported. Thus, funic reduction in the setting of continuous fetal monitoring appears to be a
reasonable approach if vaginal delivery is imminent or if cesarean delivery cannot be performed
immediately.
OUTCOME Good neonatal outcomes have been reported in the majority of patients with
cord prolapse [ 7,15,17 ]. Perinatal mortality related to cord prolapse, which was as high as 375
per 1000 births in the early 1900s, has fallen to between 36 and 162 per 1000 births within the
past few decades [ 18 ]. This decline is likely secondary to the widespread availability of
immediate delivery by cesarean birth when cord prolapse is diagnosed and significant
improvements in neonatal intensive care.

The interval between cord prolapse and delivery is a major determining factor in the immediate
neonatal outcome and perinatal mortality, but not the only factor. Team training can significantly
lower the time from diagnosis to delivery [ 19 ].
Multiple studies have evaluated the effect of diagnosis-to-delivery time on fetal outcome:

One of the largest retrospective analyses of cord prolapse (n = 132 cases) found that the
diagnosis-to-delivery interval had little influence on Apgar scores at 1 and 5 minutes if
delivery occurred within 30 minutes [ 5 ]. However, the incidence of acidemia (pH7.1)
increased after 10 minutes. There were 12 perinatal deaths, but only one of these deaths
was attributed to birth asphyxia and occurred after a 100-minute diagnosis-to-delivery
interval. The other 11 deaths were due to a variety of causes including extreme
prematurity, lethal congenital anomalies, and placental abruption, and all but one
occurred after a prolonged diagnosis-to-delivery interval (20 to 750 minutes).

In another series of 65 cases of cord prolapse, there were five asphyxiated neonates and
all five had diagnosis-to-delivery times that were shorter than the mean for the entire
group (11 versus 20 minutes) [ 17 ].

A third series of 13 patients with cord prolapse occurring in the setting of preterm
premature rupture of membranes noted 9/13 cases were associated with a delayed
diagnosis-to-delivery interval (over 60 minutes), but no neonate was acidemic (pH7.1)
at delivery [ 20 ].

On the other hand, cord prolapse occurring outside of the hospital clearly has a poor
prognosis. A series of 77 cases of cord prolapse reported 3/7 occurrences before hospital
admission resulted in fetal/neonatal death compared to no deaths among the 70 inhospital cases [ 4 ]. The in-hospital cases had a mean diagnosis-to-delivery interval of 16
min (range 8 to 35 min).

The variability in the data cited above suggests that it is not simply the diagnosis-to-delivery
interval that affects fetal outcome; the degree of cord compression also appears to be an
important factor [ 7 ]. This hypothesis is supported by rare case reports where prolonged cord
prolapse has been managed expectantly. In one such report, a patient with overt cord prolapse
diagnosed at 23 and 2/7 weeks was managed expectantly for four days due to extreme
prematurity [ 21 ]. The fetus was delivered when severe variable decelerations developed and
survived, neurologically intact at eight months follow-up.
The degree and duration of cord compression may actually be less than that predicted by simply
measuring the time interval from diagnosis-to-delivery. Time spent resuscitating the fetus in
utero (eg, by maternal position changes, bladder distention, or tocolysis) while preparations for
cesarean delivery are being made might benefit the fetus, as reflected by higher Apgar scores and
a lower incidence of neonatal asphyxia. In addition, some cords (eg, noncoiled) may be more
vulnerable to cord compression than others. Prematurity, a risk factor for cord prolapse, is a
major contributing factor to perinatal morbidity and mortality, thus gestational age should be

taken into account when citing the relative risk of mortality/morbidity. (See "Gross examination
of the placenta", section on 'Coiling' .)
There are sparse data on the long-term outcome of children whose birth was complicated by cord
prolapse. In the large series of 132 cases of cord prolapse discussed above, only 1 of 120
survivors had a long-term handicap [ 5 ]. A study that looked at risk factors for cerebral palsy
identified 110 children with cerebral palsy among 16,785 cases of cord prolapse (1 in 153 cord
prolapses) [ 22 ].
PREVENTION Patients with prematurely ruptured membranes and malpresentation are at
particular risk of prolapse and should be closely monitored. Options include checking for fetal
heart rate decelerations with continuous fetal heart rate monitoring when the patient is on the
labor unit and intermittent fetal heart rate monitoring and educating the patient regarding the
signs and symptoms of cord prolapse in other settings.
Identifying patients at risk for cord prolapse and avoiding unnecessary obstetric interventions in
these patients may prevent some cases of prolapse. However, obstetric interventions are often
indicated and cannot be avoided. Anticipation of prolapse and modification in standard
techniques can reduce the risk of prolapse in these cases. If possible, amniotomy should be
performed only when the fetal vertex is well applied to the cervix. When amniotomy is necessary
and the fetal vertex is not well applied, controlled amniotomy with either a fetal scalp electrode
or small gauge needle and simultaneous application of fundal pressure may decrease the risk of
prolapse. We suggest using a small gauge needle to puncture fetal membranes in one or more
places in the operating room when there is hydramnios or an unengaged fetal presenting part.
This "controlled amniotomy" minimizes the risk of gushing amniotic fluid and enables
emergency cesarean delivery in the event of cord prolapse.
The clinician should try to avoid disengaging the fetal presenting part when performing
procedures such as application of a fetal internal scalp electrode, intrauterine pressure catheter
insertion, fetal scalp sampling, amnioinfusion, forceps application, and manual rotation of the
fetal head.
Pregnancies with funic presentation We do not suggest routine sonography to assess
umbilical cord position. However, ultrasound examination can be used to confirm cord position
when there is a clinical suspicion of funic presentation [ 23-25 ]. Color flow Doppler studies can
clarify cord position if there is uncertainty on real-time sonographic examination ( image 1A-C )
[ 24 ].
When funic presentation is identified as an incidental finding antepartum, we suggest follow-up
evaluation of cord location to decide on the mode of delivery. In a series of 13 patients initially
identified as having cord presentation on ultrasound, three had persistent sonographic evidence
of cord presentation on follow-up and were delivered by cesarean birth, one following cord
prolapse [ 26 ]. The other four fetuses spontaneously converted to vertex with resolution of cord
presentation. There are inadequate data to recommend for or against routine scheduled cesarean
delivery if funic presentation is noted at term.

SUMMARY AND RECOMMENDATIONS

Umbilical cord prolapse is a rare event potentially associated with serious fetal/neonatal
complications. (See 'Incidence' above and 'Outcome' above.)

Risk factors for cord prolapse include (1) fetomaternal factors that lead to inadequate
filling of the maternal pelvis by the fetus and (2) iatrogenic obstetrical interventions, but
many prolapses occur without antecedent risk factors. (See 'Risk factors and etiology'
above.)

The first sign of cord prolapse is usually a severe, prolonged fetal bradycardia or
moderate to severe variable decelerations after a previously normal tracing. The prolapse
may be overt or occult. (See 'Introduction' above and 'Clinical manifestations and
diagnosis' above.)

In high risk patients, the risk of cord prolapse may be reduced by avoiding unnecessary
obstetrical interventions, controlled amniotomy, and avoiding disengagement of the fetal
head. (See 'Prevention' above.)

Standard obstetrical management of cord prolapse is prompt cesarean delivery to avoid

fetal compromise or death from compression of the cord. However, vaginal delivery may
be a reasonable option in select cases when delivery is imminent. (See 'Management'
above.)

Intrauterine resuscitation using maneuvers such as funic decompression and bladder

filling can reduce pressure on the cord while preparations are being made for delivery.
(See 'Intrauterine resuscitation' above.)

Good neonatal outcomes have been reported in the majority of patients with cord
prolapse. The interval between cord prolapse and delivery is a major determining factor
in the immediate neonatal outcome and perinatal mortality, but not the only factor. (See
'Outcome' above.)

We do not suggest routine sonography in all patients to assess umbilical cord position.
Ultrasound examination should be used to confirm cord position when there is a clinical
suspicion of funic presentation. Pregnancies with persistent funic presentation can be
delivered by cesarean, however, there are no properly controlled trials to provide a
recommended mode of delivery in this setting. (See 'Pregnancies with funic presentation'
above.)

Care of the umbilicus and management of

umbilical disorders

INTRODUCTION At birth, the umbilical cord, which provided vascular flow

between the fetus and placenta, is clamped and cut. Within the first week of life, the remnant
umbilical cord stump separates from the neonate creating the umbilicus (commonly referred to as
the navel).
Infection, hernia, granulomas, and congenital anomalies can occur in the umbilicus and are more
commonly seen in infancy.
The care of the umbilical cord and clinical problems associated with the umbilicus will be
reviewed here.
ANATOMY
Umbilical cord The umbilical cord contains two arteries and one vein, which are surrounded
and supported by gelatinous tissue called Wharton's jelly.
Thickness At birth, the average diameter and circumference of the umbilical cord in a normal
term infant is 1.5 and 3.6 cm, respectively [ 1,2 ]. Both thick and thin cords are associated with
increased risk of significant pathology. (See 'Newborn examination' below.)
Length The umbilical cord lengthens through gestation from a mean of 32 cm at 20 weeks
gestation to 60 cm at term [ 3 ]. Within each gestational age group, the length is highly variable.
As an example, in term infants born between 40 and 41 weeks gestation, the umbilical length
varies from 35 to 80 cm.
Although the cord length has no effect on fetal blood flow, both long and short cords are
associated with adverse effects:

With longer umbilical cords, there is an increased risk of cord knots, nuchal cords
(umbilical cord coiled around the neck of the fetus at the time of birth), cord prolapse,
thrombi, and fetal demise. Short umbilical cords (<35 cm) have been associated with
placental abruption and developmental abnormalities [ 4,5 ]. (See "Gross examination of
the placenta", section on 'Umbilical cord' .)

Short cords with no coiling are associated with poor fetal growth and decreased activity,
which can occur in fetuses with severe neural and musculoskeletal abnormalities.

Coiling Because the umbilical vessels are longer than the cord itself, twisting and bending of
the vessels within the cord is common. Coiling of the umbilical cord is thought to protect the
blood supply from mechanical disruption.
Although the mechanism of coiling is unknown, both excessive hypocoiling and hypercoiling are
associated with adverse perinatal outcomes. This was illustrated in a review of 885 infants [ 6 ].
The following findings were noted:

Infants with umbilical cord index (number of coils divided by the length of cord in cm)
below the 10th percentile were more likely to be premature, have a trisomic genetic
abnormality, or have a single umbilical artery. In addition, there was an increased risk of
fetal death.

Infants with umbilical cord index above the 90th percentile were more likely to have
perinatal asphyxia, umbilical arterial pH below 7.05, trisomic genetic abnormality, single
umbilical artery, or be small for gestational age.

Umbilicus The umbilicus is composed of three distinct anatomic areas ( figure 1 ):

Mamelon Area of central depression

Cicatrix Dense scar, which marks the intersection of fetal intra- and extra-embryonic
mesoderm

Cushion Slightly raised margin around the mamelon and cicatrix

Combination of these three features result in more than 60 reported normal anatomical variants [
7 ].
The umbilicus is located in the midline at the level of the iliac crest [ 8 ]. The umbilicus is lower
than usual in patients with achondroplasia, displaced superiorly during pregnancy, and inferiorly
by ascites (Tanyol's sign) [ 8 ].
Abnormal umbilical appearance or location may be a finding in a genetic disorder such as those
illustrated by the following examples [ 9 ]:

In Robinow's syndrome, a rare inherited disorder of short stature and macrocephaly with
frontal bossing, the umbilicus is high, flat, and poorly epithelialized.

In Axenfeld-Rieger syndrome, a rare genetic disorder that includes malformations of the

anterior chamber of the eye and teeth, the umbilicus is broad and prominent with a large
stalk and redundant skin. (See "Overview of glaucoma in infants and children", section
on 'Anterior segment dysgenesis' .)

In Aarskog-Scott syndrome, a disorder of multiple limb and genital abnormalities with

short stature, the umbilicus can be either flat with radiating branches of the cicatrix or a
deep longitudinally oriented ovoid depression.

EMBRYOLOGY An appreciation of the embryology of the umbilical cord helps in

understanding the pathogenesis of congenital umbilical anomalies.
In the fourth week of embryogenesis, the flat trilaminar embryonic disc folds and becomes a
cylindrical C-shaped fetus, which narrows the opening of the yolk sac to the embryo ( figure 2 ).

This narrowed opening contains the umbilical vessels, the urachus, and the omphalomesenteric
duct. The omphalomesenteric duct connects the yolk sac to the developing gut. At the same time,
the allantois, a diverticulum of the caudal hindgut, forms and becomes the urachus. The urachus
connects the developing genitourinary tract (bladder) to the umbilicus.
In normal development, both the omphalomesenteric duct and urachus involute. After involution,
no remnant of the omphalomesenteric duct persists but, in contrast, a remnant of the urachus
persists and can be seen in some individuals extending from the bladder to the umbilicus in the
preperitoneal midline.
UMBILICAL CORD AT BIRTH
Newborn examination After delivery, the clamped umbilical cord of the newborn infant is
inspected for its general appearance including the presence of discharge or a single umbilical
artery.
A thick cord may contain bowel, embryonic remnants (omphalomesenteric duct or urachal
elements), or a vascular anomaly [ 10-12 ]. Thus, evaluation of infants with a thick cord should
include histologic examination of the umbilical stump and consideration of ultrasonography to
detect embryonic remnants or bowel involvement, and cystourography to detect urologic
abnormalities [ 13 ]. Thin cords less than 1 cm in circumference are associated with infants who
are postdates or small for gestational age.
Single umbilical artery A single umbilical artery (SUA) is present in 0.2 to 0.6 percent of live
births, occurring more frequently in small for gestational age and premature infants, and twins [
14-17 ].
In infants with SUA, there is an increased rate of chromosomal and other congenital anomalies.
Multiple studies have shown that 20 to 30 percent of neonates with SUA had major structural
anomalies, often involving multiple organs [ 14-17 ]. The most commonly affected organs are the
heart, gastrointestinal tract, and the central nervous system.
SUA is an isolated finding in the remaining 70 to 80 percent of infants. In these neonates, there is
an increased incidence of occult renal anomalies. This was illustrated in a meta-analysis of seven
studies that included 204 infants who were screened for renal malformations either by
ultrasonography or intravenous pyelography [ 16 ]. A renal anomaly was detected in 16 percent
of these infants. However, the abnormality was persistent and considered clinically significant in
only one-half (8 percent). Vesicoureteral reflux (VUR) of grade 2 or higher was the major renal
finding and was found in 3 percent of the total study population.
A subsequent prospective study of infants born over six years at a single English institution
identified 137 of 33,057 live-born infants (4.1/1000) with SUA [ 18 ]. Eight infants had major
congenital anomalies (eg, complex heart disease, cloaca, anorectal, abnormal karyotypes, cystic
adenomatoid malformation, and multiple anomalies), and three had minor anomalies (eg,
preauricular skin tag, preaxial polydactyly, and syndactyly). Infants with SUA had a lower
gestational age and were more likely to be small for gestational age than control infants with two

umbilical arteries. In infants with isolated SUA, 117 of the 122 infants who underwent renal
ultrasonography had normal scans. Two infants had significant renal abnormalities: a
hypodysplastic kidney, which was already detected prenatally, and a single kidney. Renal
anomalies were more common among infants with isolated SUA compared to the control cohort
(4.1 versus 0.9 percent). There was no statistical difference in the risk of significant clinical renal
abnormalities defined but the authors (1.6 versus 0.4 percent).
Similar results were seen in a study of 65 infants with SUA from the Netherlands that reported a
clinically insignificant renal abnormality in one of 57 cases of isolated SUA [ 19 ].
Opinions differ on the value of screening these infants for renal abnormalities [ 16-18 ].
However, based upon currently available data, we do not perform further imaging for healthy
term infants with an isolated SUA, as there is a low likelihood of a renal or urological
abnormality."
Cord care In developed countries, where aseptic care is routine in the clamping and cutting of
the umbilical cord, it is unclear whether additional topical care is needed to prevent omphalitis.
This was illustrated in a meta-analysis of 10 studies from developed countries that compared dry
cord care to a number of different antiseptic topical agents including triple dye, alcohol, silver
sulfadiazine , and chlorhexidine ( table 1 ) [ 20 ]. There was no difference between dry cord care
and any of these agents in the incidence of death or omphalitis. Bacterial colonization by
Staphylococcus aureus was reduced with the use of triple dye, silver sulfadiazine , and
chlorhexidine.
In developing countries, where aseptic delivery care is not readily available, antiseptic topical
care of the umbilicus reduces the risk of omphalitis and neonatal mortality. Community-based,
cluster-randomized trials performed in Nepal, Pakistan and Bangladesh demonstrated using 4
percent chlorhexidine compared with either dry or soap/water for cord care reduced the
incidence of omphalitis and neonatal mortality [ 21-23 ].
Inappropriate cord care also can increase umbilical infections. As an example, neonatal tetanus
has been reported as a complication of inappropriate application of cow dung or bentonite clay [
24 ]. (See "Tetanus", section on 'Neonatal tetanus' .)
In summary, the care of the umbilical cord in reducing the risk of infection is dependent upon the
quality of care at delivery and postnatally. In a setting of low medical resources with a high risk
of omphalitis, the use of antiseptic cord care is a beneficial and inexpensive option that reduces
neonatal morbidity and mortality.
Cord separation Cord separation normally occurs one week after birth. This was illustrated in
a subsequent study of the previously mentioned Nepalese community-based trial that
demonstrated a mean umbilical separation time of 4.2, 4.3, and 5.3 days in infants who received
dry care, soap/water, or chlorhexidine umbilical cord care, respectively [ 25 ]. Longer time
periods to separation are associated with other antiseptic topical agents used in cord care
including salicylate sugar powder (mean 5.6 days) [ 26 ], 70 percent alcohol (mean 16.9 days) [
26 ], and triple dye (range 3 to 8 weeks) [ 27 ].

Umbilical cord separation is initiated by thrombosis and contraction of the umbilical vessels
followed by phagocyte-mediated tissue breakdown and epithelialization of the cord stump.
Stump colonization by bacteria derived from the maternal genital tract or environment occurs
soon after birth, and in some cases can cause umbilical infections. (See 'Omphalitis' below.)
Delayed separation Delayed cord separation does not have a specific definition, primarily
because of the variation in normal cord separation. In general, any cord that persists after three
weeks probably represents delayed cord separation. Delayed cord separation can be associated
with underlying immunodeficiency, infection, or urachal abnormality [ 28 ]. Neutrophil function
should be evaluated in infants with delayed cord separation and signs of umbilical infection
because infants with leukocyte adhesion defects often present with these findings. (See
"Leukocyte adhesion deficiency", section on 'LAD I' .)
Although there are no data on the care of cords that fail to separate in the normal time frame of
three weeks, these cords usually will separate without intervention eventually. Rubbing alcohol
should not be applied to the cord as it may kill bacteria that assist in cord drying and separation.
Drying of the cord may be helped by keeping the diaper folded below the cord, thereby exposing
the cord to air. In some instances, after the cord has desiccated, it may be removed by a health
care provider using a scissor or scalpel by dividing the desiccated tissue just distal to the normal
skin.
OMPHALOMESENTERIC DUCT ANOMALIES Partial or complete failure of involution
of the omphalomesenteric duct can lead to a spectrum of anomalies in the newborn infant due to
varying degrees and location of duct patency ( figure 3 ).

Complete patency results in the omphalomesenteric duct directly connecting the

umbilicus to the terminal ileum. This can lead to intestinal drainage from the umbilicus.
These infants will often appear to have a "stoma" in the umbilicus after cord separation.

Persistent tissue at the umbilicus with no intestinal connection results in an umbilical

polyp. (See 'Umbilical polyp' below.)

Persistent tissue at the ileum, with no connection to the umbilicus results in Meckel's
diverticulum. (See "Diagnostic approach to lower gastrointestinal bleeding in children",
section on 'Meckel's diverticulum' .)

Patent midduct with closure at both the umbilical and ileal ends of the
omphalomesenteric duct results in an omphalomesenteric duct cyst.

Persistent fibrous cord between the umbilicus and the ileum; the intraabdominal fibrous
band between the umbilicus and terminal ileum can lead to small bowel obstruction.

In a case series of 217 children with omphalomesenteric duct anomalies, 85 (39 percent) were
symptomatic [ 29 ]. The most common symptoms were rectal bleeding due to Meckel's
diverticulum and intestinal obstruction; abdominal pain and bilious umbilical drainage were

much less common [ 29 ]. Among the 132 asymptomatic patients, Meckel's diverticulum was
incidentally found at laparotomy.
Because more than one omphalomesenteric duct anomaly can be present, radiologic evaluation
should be performed in patients with an omphalomesenteric duct anomaly. This should include
ultrasonography and possibly a Meckel scan (99m technetium pertechnetate, which has an
affinity for gastric mucosa). If the results from ultrasonography are uncertain, computed
tomography is performed. (See "Diagnostic approach to lower gastrointestinal bleeding in
children", section on 'Meckel's diverticulum' .)
Treatment is surgical excision of the omphalomesenteric duct remnant [ 30 ].
URACHAL ANOMALIES The urachus normally involutes, resulting in a fibrous cord
between the umbilicus and the bladder. Disruption of this process can lead to a spectrum of rare
anomalies ( figure 4 ):

Complete patency results in a patent urachus with free communication between the
bladder and the umbilicus. These children generally present with a persistently wet or
draining umbilicus, and occasionally with a urinary tract infection.

Persistent tissue at the umbilicus with no connection to the bladder results in an umbilical
polyp. (See 'Umbilical polyp' below.)

Persistent tissue at the bladder with no connection to the umbilicus results in a bladder
diverticulum. Bladder diverticulum can cause ureteral obstruction at the site of bladder
insertion.

Patent midduct with closure at both the umbilicus and the bladder results in a urachal
cyst. The cyst can present as a mass, especially in older children and adults. The cyst may
become infected and present with associated signs or symptoms of abdominal pain,
erythema, or swelling, usually located below the umbilicus

In some patients, the presentation of urachal anomalies is subtle with erythema of the umbilicus
with or without drainage [ 31 ]. Pain or retraction of the umbilicus during micturition is a rare but
specific finding in patients with urachal abnormalities [ 31,32 ].
In a case series of 45 children with urachal anomalies, 19 presented with periumbilical discharge,
15 with umbilical cyst or mass, 10 with abdominal or periumbilical pain, and 1 with dysuria [ 33
]. Urachal abnormalities included urachal sinus in 22 children, urachal cyst in 16, and patent
urachus in 7.
Urachal anomalies may not be detected until adulthood. In one case series of 176 patients, which
included 130 adult patients, the most common findings at presentation for affected adults were
hematuria (49 percent), pain (27 percent), dysuria (12 percent), or the diagnosis was made
incidentally during surgery for another disorder (18 percent) [ 34 ]. In adults, half of the

pathologic specimens demonstrated evidence of adenocarcinoma. In contrast, no child had

evidence of malignancy.
In a patient suspected of having a urachal anomaly, ultrasonography is useful in diagnosis of
urachal cyst [ 35 ], and a sinogram (radiocontrast injection into urachal opening) is diagnostic for
patent urachus and urachal sinus [ 33 ]. Because of associated genitourinary abnormalities, a
renal ultrasound and voiding cystourethrogram should be obtained in all patients with urachal
anomalies.
The treatment of urachal anomalies is surgical excision of the entire lesion including a cuff of the
normal bladder. Complete excision is important to eliminate the risk of future urachal
adenocarcinoma. (See "Nonurothelial bladder cancer", section on 'Urachal adenocarcinoma' .)
UMBILICAL HERNIA The fascial opening (umbilical ring) exists to allow passage of the
umbilical vessels from the mother into the fetus. After birth, this fascial opening closes
spontaneously with continued growth of the rectus abdominis muscles toward one another.
Ultimately, complete closure occurs with fusion of the peritoneal and fascial layers within a
small fibrous area of the umbilicus. Closure of the umbilical ring is complete in almost all
children by 5 years of age, but may be slower in black children [ 36,37 ].
Although closure is complete in almost all children by five years of age, closure can continue in
older children as manifested by lower rates of umbilical hernia [ 37,38 ]. This was illustrated in a
cross-sectional study of 665 black children between 4 and 11 years of age that demonstrated the
following rates of umbilical hernia based upon age [ 37 ].

4 to 5 years of age (n = 51) 14 percent

6 to 7 years of age (n = 142) 4 percent

8 to 9 years of age (n = 221) 3 percent

10 to 11 years of age (n = 251) 2 percent

Umbilical herniation in adults is discussed separately. (See "Overview of abdominal hernias",

section on 'Umbilical hernia' .)
Spontaneous closure is less likely to occur in patients who have an opening that is greater than
1.5 cm, a significant amount of protruding skin, are older, or have an underlying predisposing
condition [ 32,39 ]. Umbilical hernias are frequently seen in patients with Ehlers-Danlos [ 40 ],
Beckwith-Wiedemann syndrome [ 39 ], Down syndrome, mucopolysaccharidoses [ 41 ],
hypothyroidism [ 42 ], or trisomy 18. Increased intraabdominal pressure from ascites or
peritoneal dialysis also can prevent closure of the umbilical ring resulting in herniation [ 43 ].
Clinical findings Although the vast majority of pediatric patients with umbilical hernias are
asymptomatic, in rare cases the hernia can interfere with feeding, especially in young infants
with hernias that contain bowel.

Umbilical hernias are detected during the newborn abdominal examination, particularly when
there is increased intraabdominal pressure from crying. Umbilical hernias are easily reduced
even if they are quite large, and the borders of the fascial defects can be palpated through the
skin. The fascial defect, not the degree of protrusion, is most indicative of whether spontaneous
closure will occur. It is important to differentiate umbilical hernias from the less common
abdominal wall (also called ventral or supraumbilical) hernias that do not close spontaneously [
44 ].
In children, umbilical hernias rarely become incarcerated (inability to be reduced by
manipulation) [ 45 ] or strangulated (vascular compromise of the contents of an incarcerated
hernia) [ 46 ], or even more rarely rupture.
Management Because the natural course of the umbilical ring is eventual closure, most
umbilical hernias will spontaneously resolve. In general, asymptomatic children with an
umbilical ring that continues to decrease can be observed. Surgical intervention is required only
in a minority of patients.
Incarcerated umbilical hernia in children is an absolute indication for surgical repair to avoid
strangulation. Children with large, proboscoid (trunk-like) hernias ( picture 1 ) without any
decrease in the size of the umbilical ring defect over the first two years of life, generally require
surgery, because their hernias will fail to close spontaneously. Other relative indications for
surgical repair include defects that cease decreasing in size, are symptomatic, or create
significant behavioral problems.
UMBILICAL MASSES
Infants Umbilical masses in the neonate include umbilical granuloma, polyp, or ectopic
tissue.
Umbilical granuloma In neonates, umbilical granuloma is a common abnormality and is the
most common cause of an umbilical mass. It is a soft, moist, pink, pedunculated, friable lesion of
granulation tissue that varies in size from 3 to 10 mm in length ( picture 2 ).
Umbilical granuloma forms in the first few weeks of life from excess tissue that persists at the
base of the umbilicus after cord separation [ 47 ]. Granuloma formation is more likely to occur
when there is inflammation of the umbilical cord usually due to infection, which also delays cord
separation.
Umbilical granuloma is most often detected after the cord has separated because of persistent
drainage of serous or serosanguinous fluid or moisture around the umbilicus.
The most common treatment for umbilical granuloma is topical 75 percent silver nitrate , usually
applied by a wooden applicator with premounted silver nitrate. The lesion is treated once or
twice a week for several weeks, but generally only a few applications are required for successful
treatment. Caution should be exercised in applying silver nitrate because it can cause chemical
burns or staining of the surrounding skin.

In cases that fail to respond to topical silver nitrate , ligation can be performed in the office
without discomfort [ 48 ]. Before ligation, the umbilicus should be carefully examined to rule out
other causes of umbilical masses, such as umbilical polyp. Failure of the granuloma to resolve
with ligation and/or silver nitrate should also increase the suspicion that the lesion is actually an
umbilical polyp.
Umbilical polyp Umbilical polyps are firm masses comprised of intestinal epithelium or
uroepithelium, which are omphalomesenteric ductal or urachal embryologic remnants [ 49,50 ].
Umbilical polyps are rare and often larger than granulomas, do not respond to silver nitrate
therapy, and require surgical excision.
If there is any question of whether an umbilical mass in a neonate is a polyp or granuloma,
histopathologic evaluation of the lesion should be performed. If a polyp is diagnosed, further
evaluation for associated embryologic anomalies (eg, Meckel's diverticulum) should be
performed. (See 'Omphalomesenteric duct anomalies' above and 'Urachal anomalies' above.)
Ectopic tissue Ectopic tissue in the umbilical cord is a rare lesion that presents as a solid
mass. Ectopic tissue can include pancreas, which most likely arises from growth of pluripotent
cells derived from the omphalomesenteric duct [ 32,51 ], or liver, which probably results from
mechanical entrapment as the umbilical ring closes [ 32,52 ]. Surgical excision is required for its
removal.
Older children and adults Umbilical masses in older children and adults are uncommon. Both
benign and malignant umbilical tumors have been reported [ 32 ].

Benign lesions include hamartomas, pyogenic granulomas, nevi, inclusion cysts,

hemangiomas, dermatofibromas, neurofibromas, granular cell tumors, desmoid tumors,
and lipomas.

Primary malignancies are rare and include melanoma, urachal adenocarcinoma,

squamous cell carcinoma, and basal cell carcinoma.

Metastatic lesions have been reported from many primary sources (eg, stomach, pancreas,
endometrium, ovary, cervix, colon, small bowel, gallbladder, prostate, lung, and breast).
Metastatic lesions to the umbilicus are called "Sister Mary Josephs node", named after
the nun who worked with Dr. William Mayo, the surgeon who developed the surgical
approach to umbilical hernia repair [ 53 ].

Other umbilical lesions include omphalitis, which result from lint accumulation in the umbilicus
that become "ossified". These lesions are often hard and black, and thus can be misdiagnosed as
a melanoma. In addition, keloid formation within or near the umbilicus can occur and mimic a
primary tumor.
OTHER ABNORMALITIES

Appendicoumbilical fistula Appendicoumbilical fistula is rare and appears to occur in two

settling: during development with entrapment of the appendix in the closing umbilical ring; and
after a perforated appendicitis. The presenting manifestation is drainage of stool from the
umbilicus.
Enteric fistula Enteric fistula to the umbilicus has been reported in patients with Crohn's
disease or following surgery for tuberculous peritonitis [ 8,54 ]. (See "Clinical manifestations of
Crohn's disease in children and adolescents" .)
UMBILICAL INFECTION Umbilical infections, which can progress to systemic infections,
occur primarily in the newborn because of the following predisposing factors:

Immediately following birth, the umbilicus becomes colonized with a diverse flora of
microorganisms. Staphylococcal species and other gram-positive cocci are present within
hours, and enteric organisms follow shortly thereafter [ 55-57 ].

Devitalized tissues of the umbilical cord stump provide an excellent growth medium for
bacteria.

The thrombosed blood vessels within the umbilical cord stump provide an entry for
microorganisms into the bloodstream of the neonates potentially leading to sepsis.

Omphalitis Omphalitis is an infection of the umbilicus and/or surrounding tissues. It is

predominantly a disease of the neonate and is characterized by purulent discharge from the
umbilical cord stump with surrounding induration, erythema, and tenderness ( picture 3A-B and
picture 4 ). Umbilical stump bleeding may occur with omphalitis as a result of delayed
obliteration of the umbilical vessels.
Omphalitis is rare in developed countries with a reported incidence of 0.7 percent [ 58 ].
However, in the developing world, the estimated incidence is as high as 6 percent [ 59 ].
In the newborn, risk factors for the development of omphalitis include low birth-weight,
prolonged labor, prolonged rupture of membranes or maternal infection, non-sterile delivery,
umbilical catheterization, and home birth [ 59-61 ]. Improper cord care also increases the risk of
omphalitis, such as cultural application of cow dung. Abnormalities of the immune system, such
as defects in leukocyte adhesion, neutrophil or natural killer lymphocyte function, and interferon
production, can contribute to the development of omphalitis [ 60,62 ].
Systemic signs, including lethargy, fever, irritability, and poor feeding are suggestive of more
severe infection or complication. The most common complication of omphalitis is sepsis [ 61 ].
Other complications include septic umbilical arteritis, portal vein thrombosis, liver abscess,
peritonitis, intestinal gangrene, small bowel evisceration, necrotizing fasciitis, and death [ 12,55
].
Among infants with omphalitis, mortality rate is estimated between 7 to 15 percent [ 63 ]. Male
sex, prematurity, septic delivery (including unplanned home delivery), and abnormal temperature

are reported risk factors for poor prognosis in infants with omphalitis. However, data are limited
and firm conclusions cannot be drawn regarding the role of these factors in mortality [ 63 ].
Although rare, the development of necrotizing fasciitis is associated with a higher mortality rate.
(See 'Necrotizing fasciitis' below.)
Omphalitis is a polymicrobial infection. Historically, the predominant pathogens included
Staphylococcus aureus, Streptococcus pyogenes, and gram-negative bacteria such as Escherichia
coli, Klebsiella pneumoniae, and Proteus mirabilis [ 64,65 ]. However, with the routine use of
antistaphylococcal cord care regimens, gram-negative infections of the umbilicus have increased
[ 61,66 ]. In addition, anaerobic bacteria such as Bacteroides fragilis, Clostridium perfringens,
and Clostridium tetani can contribute to umbilical infections, especially in infants born to
mothers with chorioamnionitis [ 67 ]. In these infants, foul-smelling umbilical drainage is a
typical finding.
Whenever possible, cultures of the discharge should be obtained prior to the start of antibiotic
therapy. Blood and cerebrospinal fluid cultures should also be obtained in infants with systemic
signs (eg, fever) as they are more likely to be septic or develop meningitis.
Antibiotic treatment of omphalitis is required and is directed against gram-positive and gramnegative organisms [ 61 ]. In the neonate, parenterally administered antistaphylococcal penicillin
and aminoglycoside agents are administered to decrease the risk of significant complications,
such as sepsis and necrotizing fasciitis. In communities with a high prevalence of methicillinresistant S. aureus, vancomycin should be used in place of an antistaphylococcal penicillin.
Clindamycin or metronidazole also has been suggested in the treatment of infants with
omphalitis for anaerobic coverage, especially those with foul smelling discharge or born to
mothers with amnionitis [ 47,67 ]. We typically administer a 10-day course of intravenous
antibiotics in neonatal patients, which can be modified dependent upon the patient's clinical
response and whether complications develop.
In older patients, similar antibiotic coverage can be administered orally, and is modified based on
culture results and clinical improvement.
Mild discharge from the umbilical stump in the absence of inflammatory signs may be a normal
occurrence, even when accompanied by some odor. Some clinicians treat infants with minimal
symptoms with topical applications such as alcohol, bacitracin , or mupirocin . However, there is
no evidence of efficacy of this practice or on the efficacy of the administration of oral antibiotics
in these infants.
In the developing world, antiseptic topical care of the umbilicus stump reduces the risk of
omphalitis and neonatal mortality. (See 'Cord care' above.)
Necrotizing fasciitis Neonatal necrotizing fasciitis is a rare complication of omphalitis [ 47 ].
It is a polymicrobial infection of the skin, subcutaneous fat, and superficial and deep fascia. It is
characterized by rapid spread of infection and inflammation, and signs of systemic toxicity.
Infants presenting with fasciitis have a high incidence of bacteremia, shock, and death [ 47,61 ].
Reported mortality rates are as high as 60 to 85 percent [ 32,68 ]. Prompt aggressive surgery,

broad-spectrum antibiotics, and supportive care are critical in the management of necrotizing
fasciitis [ 32 ]. (See "Necrotizing soft tissue infections" .)
Funisitis Funisitis is inflammation of the umbilical cord that occurs with chorioamnionitis (in
response to intraamniotic infection). Funisitis involves only the external surface of the cord and
Wharton's jelly and does not involve the umbilical vessels. The inflammatory cells seen
migrating through the fetal vessels in the cord are evidence of a fetal response to an external
pathologic factor that is maternal in origin.
Funisitis does not involve the umbilical stump. Necrotizing funisitis occurs with long-standing
infection and is characterized by inflammatory debris and calcification of umbilical cord tissues.
Infants with funisitis can be born healthy, but should be treated with broad-spectrum antibiotics
(similar to the regimen used for omphalitis) for a minimum of seven days of therapy. (See
"Histopathology of placental disorders" and "Placental infections" .)
Infected umbilical piercing Infected umbilical piercings are characterized by purulent
discharge from the pierced skin with surrounding induration, erythema, and tenderness.
Local wound care and topical antibiotics usually are sufficient for resolution of local infection in
older children, and it is usually not necessary to remove the device. When infection persists or
occurs in a child younger than 1 year of age, oral antibiotics aimed at treating S. aureus and S.
pyogenes are recommended. Neonates with an infected umbilicus require an evaluation for
sepsis and intravenous antibiotics. (See 'Omphalitis' above and "Body piercing in adolescents
and young adults" .)
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