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PEDIATRIC SURGERY

ISBN: 978-0-323-07255-7
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Library of Congress Cataloging-in-Publication Data

Pediatric surgery. 7th ed. / editor in chief, Arnold G. Coran ; associate editors, N.
Scott Adzick . . . [et al.].
p. ; cm.
Includes bibliographical references and index.
ISBN 978-0-323-07255-7 (2 vol. set : hardcover : alk. paper)
I. Coran, Arnold G., 1938- II. Adzick, N. Scott.
[DNLM: 1. Surgical Procedures, Operative. 2. Child. 3. Infant. WO 925]
617.98dc23
2011045740

Editor: Judith Fletcher

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Printed in the United States of America

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CHAPTER 74

Disorders
of the Umbilicus
Robert E. Cilley

recommended, and the importance of preservation of the

appearance of the umbilicus rather than excision was emphasized. Repair of umbilical abnormalities was recognized as
formidable in small children, and little is known of the true
operative morbidity and mortality in the hands of the surgical
pioneers who first attempted their correction.
The most complete work on the umbilicus is the classic text
by Cullen, published in 1916.2 This encyclopedic work is still
the most definitive work on the subject. Cullens curiosity was
originally stimulated by a case of cancer at the umbilicus, and
it inspired him to explore the entire topic of umbilical pathology. He stated, The study of the umbilicus, which in the
beginning had seemed so unimportant, became so fascinating
that I covered most of the literature on the subject.2
The vital functions of the umbilicus in utero and the structures that pass through it in normal development contrast with
its lack of physiologic importance after birth. Its psychologic
importance throughout life is attested to by individuals who
have endured surgical loss of their umbilicus. Pediatric surgeons are the first to be consulted whenever there is an
unusual finding of the umbilicus in newborns and older
children. Umbilical herniorrhaphy is among the more commonly performed operations in childhood. In addition, the
umbilicus serves as a portal of entry for most laparoscopic procedures, and it may be used as an intestinal or urinary stoma
site. Cannulation of its vessels, either in their native location or
transposed surgically, provides vascular access in neonates.
The umbilicus is considered to be aesthetically important,3
and it may be an object of display and adornment. Exposure
of the umbilicus is commonplace, as is the use of jewelry and
piercings to enhance its appearance.

Normal Embryology
------------------------------------------------------------------------------------------------------------------------------------------------

History
------------------------------------------------------------------------------------------------------------------------------------------------

Umbilical malformations have been depicted in art and sculpture since antiquity, but the developmental basis for these
abnormalities was not recognized until the late nineteenth
century. Surgical textbooks, such as that by von Bergmann
in 1904, clearly describe the embryology responsible for persistence of the vitellointestinal duct as a fistula, sinus, or cyst.1
The symptoms of fecal drainage (congenital umbilical anus)
and prolapse of the intestine were well known. The surgeon
was advised to avoid pitfalls such as excision of an umbilical
tumor that exposed two intestinal lumens because it would
indicate that the vitellointestinal remnant had been excised
in excess back to the ileum. An umbilical polyp representing a persistent remnant of the duct was referred to as an
enteroteratoma.
Surgical management has changed little in the past 100 years.
Interestingly, then as now, granulomas of the umbilical cord
were treated by silver nitrate cauterization. The embryologic
basis of developmental abnormalities of the urachus was similarly recognized, and their surgical treatment was described
much as it is today. The natural history of spontaneous resolution of most umbilical hernias was also understood at the end
of the nineteenth century. External compression was often

The classic description of the formation of the umbilicus

indicates that the abdominal wall forms by a combination of
lateral infolding and ventral flexion of the disk-shaped trilaminar embryo that begins in the fourth gestational week. However, the actual growth of the embryo does not truly involve
bending and folding of structures but rather represents
differential growth of tissues. Initially, the amnion is located
in a dorsal direction, whereas the yolk sac occupies a ventral
position. The embryo is attached to the chorion, the forerunner of the placenta, by a connecting stalk composed of extraembryonic mesoderm in which the umbilical vessels develop
and into which the allantois grows (Fig. 74-1, A). The yolk sac
maintains its ventral position but is divided into intracoelomic
and extracoelomic portions (Fig. 74-1, B). The intracoelomic
portion, derived from the roof of the yolk sac, becomes the
primitive alimentary canal and maintains a connection with
the extracoelomic portion through the vitelline or omphalomesenteric duct. This connection is normally lost by the fifth
to seventh week of gestation.2,4 Persistence of this connection,
as a remnant of either the developing alimentary tract or the
accompanying vitelline vessels, accounts for some of the
abnormalities described in this chapter.
Early in the third week of gestation, a diverticulum called
the allantois forms from the posterior wall of the yolk sac
and extends into the connecting stalk of the embryo
(Fig. 74-1, A and B). The allantois serves as a reservoir for
the developing renal system in lower vertebrates but has no
961

962

PART VII

ABDOMEN

FIGURE 74-1 A, A 1.7-mm embryo (third week). The primitive gut is not yet separate from the yolk sac. The amniotic cavity can be seen dorsally.
The umbilical vessels develop in the extraembryonic mesoderm and connect the embryo to the developing placenta. B, A 2.5-mm embryo (fourth week).
Infolding and flexion of the embryo draw the amnion around the body. The omphalomesenteric duct is part of the developing umbilical cord. (From Cullen
TS: Embryology, Anatomy, and Diseases of the Umbilicus Together with Diseases of the Urachus. Philadelphia, WB Saunders, 1916.) C, A 5-mm embryo
(fifth week) demonstrating a complete umbilical cord. The omphalomesenteric duct connection between the yolk sac and the alimentary tract is lost
between the fifth and seventh weeks. D, A 45-mm embryo (10 weeks) viewed from inside. The intestines, which were extraembryonic coelomic (i.e., within
the umbilical cord) between the sixth and tenth weeks, have returned to the peritoneal cavity.

CHAPTER 74

known role in human development; it remains rudimentary as

the transitory extraembryonic portion of the urachus. As the
distal hindgut, or cloaca, partitions into the urogenital sinus
ventrally and the anorectal canal dorsally, the developing bladder remains connected by the urachus to the allantois within
the body stalk (see Fig. 74-1, D). The urachus is derived
wholly from the developing bladder and may persist in various
forms, which accounts for the abnormalities described later.
As the embryo develops, the amnion is drawn around it to
surround the embryo and cover all the developing umbilical
cord structures including the allantois, umbilical vessels, vitelline duct, and primitive mesenchymal tissue (Wharton jelly)
(Fig. 74-1, C). During the period of rapid intestinal growth
between the sixth and tenth weeks of gestation, the developing midgut is extracoelomic. As the body wall continues to
develop, the intestines are incorporated into the coelomic
cavity and intestinal rotation and fixation progress. The fibromuscular umbilical ring continues to contract and is nearly
closed by the time of birth (Fig. 74-1, D). Persistence of the
fascial opening as an umbilical hernia occurs frequently and
is commonly seen in premature infants. Unlike other abdominal wall defects, umbilical hernias tend to resolve without
specific treatment as a result of the ongoing development of
tissues at the umbilical ring after birth. The fate of the structures that relate to the development of the umbilicus is shown
in Table 74-1.

Umbilicus at Birth
------------------------------------------------------------------------------------------------------------------------------------------------

Modern obstetric practice uses plastic clamps that are placed a

few centimeters from the umbilical skin during cord division
at the time of delivery. Topical antimicrobials such as triple
dye, bacitracin, silver sulfadiazine, povidone-iodine, chlorhexidine, hexachlorophene, alcohol, salicylic sugar powder,
green clay powder, silver-benzyl-peroxide powder, and 1%
basic fuchsin may be applied to the cord after birth.5,6 All
these agents are effective in reducing bacterial colonization
rates, and their use is recommended when adequate cord care

DISORDERS OF THE UMBILICUS

963

cannot be guaranteed. These agents may affect cord separation

time, some cause discoloration, and repeated use of iodinecontaining antimicrobials may result in systemic absorption
of iodine and suppression of thyroid function. There is good
evidence that, in developed countries, dry cord care, without
the application of topical antibiotics, in association with routine soap and water bathing and meticulous hand washing
practices is as effective as topical agents in reducing infection.7
However, a recent randomized prospective study demonstrated fewer cord-related complications in infants treated
with chlorhexidine powder compared with standard dry
cord care.8 In the undeveloped world, antiseptic cord cleansing with chlorhexidine may significantly reduce neonatal
morbidity and mortality.9
Intestinal injury may result from injudicious placement of an
umbilical cord clamp when an unrecognized small hernia of the
umbilical cord (i.e., a small omphalocele) is present. Abdominal
wall defects that relate to the umbilicus (i.e., gastroschisis and
omphalocele) are covered in Chapter 75.
The normal time for separation of the umbilical cord after
birth ranges from 3 days to 2 months.10 Antimicrobial treatment may prolong cord separation by decreasing leukocyte
infiltration. Delayed separation of the umbilical cord has been
associated with heritable neutrophil mobility defects and
widespread infections that are often lethal.11 The abnormal
neutrophils lack a membrane glycoprotein, which results
in abnormal attachment, chemotaxis, and phagocytosis.10
Although persistence of umbilical cord attachment beyond
3 weeks of age has been suggested to be a sign of such immunologic abnormalities, recent studies that have included more
than 600 newborns have demonstrated the range of normal
newborn cord separation to be broad (3 to 67 days), with a
mean of 14 to 15 days.10,12 In these studies, nearly 10% of
normal newborns underwent cord separation after 3 weeks
of age, thus indicating that delayed cord separation is not a
reliable indicator of immunologic disease. If prolonged cord
separation is associated with umbilical infection, leukocyte
adhesion deficiency disorders should be suspected and an
immunologic evaluation performed.6,13,14

TABLE 74-1
Fate of Structures Related to the Developing Umbilicus
Structure

Fate

Remnants, Pathologic Condition

Urachus (connects the bladder

to the allantois)
Omphalomesenteric duct
(connects the midgut to the
yolk sac)
Omphalomesenteric arteries

Obliterates

Median umbilical ligament, patent urachus,

sinus, cyst
Meckel diverticulum, patent omphalomesenteric duct,
sinus, cyst, bands, polyp

Omphalomesenteric veins

Umbilical arteries
Umbilical veins

Obliterates

Most regress; fuse to form the celiac, superior mesenteric,

and inferior umbilicus
Plexus around the duodenum becomes the superior
mesenteric and portal vein (contribution from both
the left and right vein)
Obliterate after birth
Right obliterates; left returns placental blood to
the inferior vena cava through the ductus venosus

Dominant artery may accompany Meckel diverticulum,

fibrous band to the mesenteric arteries
Preduodenal portal vein if the ventral portion
of the plexus persists
Medial or lateral umbilical ligaments*
Falciform ligament

*Atlases and anatomy texts variably refer to the obliterated umbilical arteries as the medial or lateral umbilical ligament. When called the medial umbilical ligaments,
the epigastric vessels are called the lateral umbilical ligaments. When called the lateral ligaments, the epigastric vessels are referred to as the epigastric folds.

964

PART VII

ABDOMEN

After separation of the cord, the umbilicus may have many

appearances. A normal umbilicus is characterized by a depression in which may be found the mamelon (a central eminence
that contains the remnants of the solid portion of the umbilical
cord) and the cicatrix (dense scar where the intraembryonic and
extraembryonic coelom were in continuity). The cushion is the
slightly raised margin that surrounds the umbilical depression.
Cullen described more than 60 normal configurations of the
umbilicus.2

Umbilical Abnormalities
------------------------------------------------------------------------------------------------------------------------------------------------

ACQUIRED
Umbilical Granuloma
After cord separation, a small mass of granulation tissue may
develop at the base. These granulomas consist of true granulation tissue with fibroblasts and abundant capillaries; the
granulomas range in size from 1 mm to approximately 1 cm.
The surface often has a pedunculated appearance. Umbilical
granulomas may be treated by cauterization with one or more
applications of silver nitrate until the area epithelializes. Alternatively, the granuloma may be excised and silver nitrate or
absorbable hemostatic material applied.15 If the mass does
not respond to cauterization, a true umbilical polyp or sinus
tract must be suspected (see later). Care must be taken with
silver nitrate application because burns and skin injury may
occur.16
Umbilical Infections
Although modern perinatal practice has dramatically reduced
the incidence of omphalitis, infections of the umbilicus still
occur with alarming morbidity and mortality, particularly in
undeveloped countries.17 Rigorous asepsis, hand washing,
and cord care (either dry cord care or topical antimicrobials)
have reduced the incidence of umbilical infections to less than
1% in hospitalized newborns.18 Before the institution of
such practices, the mortality rate for omphalitis was 65%.
The primary pathogens implicated in these infections were
Staphylococcus aureus and Streptococcus pyogenes. Currently,
gram-negative bacteria play an important role in the pathogenesis of umbilical infections. Severe infections are often
polymicrobial. Omphalitis may be manifested as a purulent
umbilical discharge or periumbilical cellulitis. Delivery at
home, low birth weight, use of umbilical catheters, and septic
delivery are risk factors. Tetanus infection occurs on rare
occasions. Intravenous antibiotic therapy is effective in eradicating most infections. Omphalitis is a common problem in
developing countries, where it accounts for more than a
quarter of neonatal hospital admissions.19,20
Cellulitis may progress to fasciitis, and such progression
may be subtle. Signs of necrotizing fasciitis include abdominal
distention, tachycardia, purpura, blistering, pyrexia, hypothermia, leukocytosis, and progression of cellulitis despite
antibiotic therapy. Bacteriologic cultures demonstrate polymicrobial flora.21 Necrotizing fasciitis and umbilical gangrene
may be lethal and require immediate wide surgical debridement for patient survival.18,2227 Excision should be performed immediately on recognition; all infected skin, fat, and
fascia should be excised back to viable, bleeding abdominal wall musculature. The umbilicus is obligatorily excised.

Excision of preperitoneal tissue including the umbilical vessels

and urachal remnant may be critically important to achieve
eradication of the infection because these tissues harbor invasive
bacteria and may provide a route for the progressive spread of
infection seen after less extensive surgical debridement.22 The
defect may require a temporary prosthetic patch for closure,
but ultimate fascial closure and umbilical reconstruction may
leave an acceptable appearance. Hyperbaric oxygen therapy
has been advocated as adjuvant therapy, but it is not of proven benefit.26 The overall reported mortality associated with
necrotizing fasciitis in collected series is 81%.18,2227
Umbilical drainage resulting from chronic infection of
umbilical remnants such as umbilical artery remnants has also
been reported.28 Excision and debridement are curative.
Omphalitis can result in necrosis and breakdown of the
umbilical stump with spontaneous evisceration within the
first 2 months of life and may be associated with portal venous
thrombosis and subsequent extrahepatic portal hypertension.

CONGENITAL
Omphalomesenteric Remnants
Remnants of the vitelline or omphalomesenteric duct account
for a wide variety of umbilical abnormalities that may require
surgical correction.28a These remnants include fistulas, sinus
tracts, cysts, mucosal remnants, and congenital bands. Typical variations of the pathologic varieties are illustrated in
Figure 74-2, A to F.2,29,30
If the omphalomesenteric duct is patent from the terminal
ileum to the umbilicus, fecal umbilical drainage will be noted
(Fig. 74-3, A). Although this event is dramatic to parents, the
problem is immediately recognizable on examination and
parents may be reassured that prompt surgical correction is
curative. Prolapse of the proximal and distal ileum through
the patent duct has a characteristic appearance. Although contrast injections are of interest, they do not change the surgical
approach (Fig. 74-3, B). Anatomically unusual conditions
such as an unexpected origin of the omphalomesenteric duct
from the appendix will be recognized at the time of operation
(Fig. 74-4).31,32 Unless another, more serious medical condition exists, a patent omphalomesenteric duct should be
excised promptly. A mechanical intestinal preparation is not
necessary, although we customarily stop formula feeding;
perioperative intravenous antibiotics are also given. The operation may be performed through the umbilicus itself or
through an incision below the umbilicus. Full exploration
and identification of all umbilical structures including one
vein, two arteries, and the urachal remnant are indicated.
The omphalomesenteric duct is traced to the ileum and
divided. The ileum is closed, and care must be taken to control
any dominant vitelline vessels that may be present. After the
fascia is closed, umbilicoplasty is performed.
Small duct remnants and sinuses may have less characteristic drainage. Injection of contrast material may be helpful in
delineating the nature of the problem in these instances, but
surgical exploration remains the definitive diagnostic test. It
is important that a full exploration is performed and that all
umbilical structures including the intraperitoneal undersurface
of the umbilicus are visualized to identify and remove any
bands attached to the small intestine. If a Meckel diverticulum
is attached to an omphalomesenteric band discovered at exploration, it is excised. Cystic remnants of the omphalomesenteric

CHAPTER 74

DISORDERS OF THE UMBILICUS

965

FIGURE 74-2 Various omphalomesenteric duct remnants. A, Umbilical cyst containing intestinal tissue. B, Umbilical sinus with a band. C, Umbilical polyp
covered with intestinal mucosa. D, Fibrous band containing a cyst. E, Meckel diverticulum. F, Patent omphalomesenteric duct. Other varieties and
combinations exist.

FIGURE 74-3 A, This photo of a newborn demonstrates probe patency of an omphalomesenteric duct into the ileum. B, A radiograph with contrast
medium injected into a patent omphalomesenteric duct demonstrates filling of the small intestine. Studies of this sort are not usually necessary.

duct may become infected and cause acute symptoms, even in

older individuals. If an abscess has formed, it may require surgical drainage; excision of any remnant may be accomplished
at a later time. The omphalomesenteric duct or any remnant
attachments between the abdominal wall and the intestine

may cause angulation, volvulus, or herniation of intestinal loops,

thereby resulting in mechanical intestinal obstruction. The
nature of the obstruction will be discovered during laparotomy.
Rarely, spontaneous regression of a patent omphalomesenteric duct may occur.33,34 In one case the defect was

966

PART VII

ABDOMEN

FIGURE 74-4 An unusual omphalomesenteric duct in continuity with

the appendix. Presentation was as a large umbilical polyp. Correction
was performed through the umbilicus.

documented by a fistulogram shortly after birth, but it was not

operated on until the patient was 3 months of age. At that
time, only a Meckel diverticulum was found, but it had no
connection to the umbilicus, thus indicating that some regression had occurred in the interim. The Meckel diverticulum
and its treatment are discussed in Chapter 84.
Urachal Remnants
2,35,36

Various abnormalities of the urachus have been described.

The typical abnormalities are depicted in Figure 74-5. A patent
urachus is associated with drainage of urine from the umbilicus. Clear drainage from the umbilicus should always raise
suspicion of a patent urachus. Although the definitive anatomy
is discovered during laparotomy, frank drainage of urine from
the umbilicus requires an investigation of the urinary tract
to look for bladder outlet obstruction in which the urachus
is functioning as a relief valve (Fig. 74-6).37 Such conditions
are rare. A patent urachus may be approached either through
the umbilicus or through an infraumbilical incision. It is

FIGURE 74-6 Radiograph with contrast medium injected into a patent

urachus demonstrates filling of the bladder. (From Jona JZ: Umbilical
anomalies. In Raffensberger JG [ed]: Swensons Pediatric Surgery, 5th ed,
Norwalk, Conn, Appleton & Lange, 1990. Used with permission.)

important to identify all the umbilical structures for a definitive

diagnosis. The patent urachus is ligated and transected at the
level of the bladder; broad-based connections are closed in two
layers with absorbable sutures. Excision of urachal remnants
using laparoscopic techniques has been described.38,39
Urachal sinuses may give rise to umbilical drainage or be
discovered on examination. Urachal cysts most often cause
an infection manifested as a painful mass localized between

FIGURE 74-5 Various urachal remnants. A, Patent urachus with communication between the bladder and umbilicus. B, Urachal sinus. C, Urachal cyst,
which is usually associated with infection.

CHAPTER 74

the umbilicus and the suprapubic area. Ultrasonography or

computed tomography may be helpful to confirm the diagnosis. Other unusual manifestations have been reported including a lateral mass.40 The urachus has also been described as
exiting from the midline below the umbilicus.41,42 In addition, a patent urachus may be one of the causes of a giant umbilical cord in the newborn.43 When a urachal cyst becomes
infected and develops into an abscess, drainage of the acute
process is required. After the abscess is drained, complete
healing may take place. It is unknown whether subsequent
operation to remove any residual cyst remnants is necessary.
Urachal remnants may cause complications later in life. Abnormal epithelium including colonic, small intestine, and
squamous may be present in incidentally removed urachal
remnants.44 The fate of these tissues is unknown, but many
different malignant tumors have been reported to originate
from the urachus. A partial list of tumors in adults arising from
the urachus is shown in Table 74-2. Pediatric tumors including rhabdomyosarcoma and neuroblastoma may originate
from urachal remnants as well.45,46
Pain plus retraction of the umbilicus during micturition has
been described as a sign of a urachal anomaly.47,48 Resection of
the urachal remnant is curative.
Diagnostic imaging including ultrasound, contrast injections, computed tomography, and magnetic resonance imaging
may occasionally be helpful in diagnosing and treating umbilical abnormalities.49 An infant with umbilical discharge caused
by both a persistent urachus and an omphalomesenteric duct
has been reported.50
Umbilical Dysmorphology
A single umbilical artery may occur in conjunction with many
syndromes and is associated with congenital abnormalities in
a third of cases. Such abnormalities include trisomy 18 and
renal and cardiac anomalies. Children with dysmorphic features may have characteristic findings that aid in diagnosis.
Minor abnormalities that lack medical significance can nonetheless provide insight into the nature and timing of dysmorphologic events that occur during development.51 Commonly,
dermatoglyphics, hair patterning, auricular shape, and genital
configuration are part of such observations. Minor abnormalities of the configuration of the umbilicus may be useful in the
classification of dysmorphologic findings. For example, an
umbilicus that is situated unusually high on the abdominal
wall at the level of the lower rib cage and is flat and poorly
epithelialized indicates Robinow syndrome, which is also
characterized by a flat facial profile, mesomelic shortening,
TABLE 74-2
Tumors Arising from the Urachus
Adenocarcinoma
Transitional cell carcinoma
Squamous cell carcinoma
Mucinous (cyst) adenocarcinoma
Malignant fibrous histiocytoma
Fibrosarcoma
Pleomorphic sarcoma
Yolk sac tumor
Inflammatory pseudotumor
Villous adenoma (premalignant)

DISORDERS OF THE UMBILICUS

967

and genital hypoplasia. If the umbilicus is broad and prominent with a large stalk and redundant periumbilical skin,
Rieger syndrome should be suspected, especially if these
umbilical abnormalities occur in conjunction with goniodysgenesis and hypodontia. If the umbilicus is prominent with
a button-like central portion in a deep longitudinally oriented ovoid depression or flat with radiating branches of the
cicatrix, Aarskog syndrome, a condition classically characterized by short stature, facial dysplasia, syndactyly, and genital
anomalies, is indicated.52

OTHER CONGENITAL AND ACQUIRED

PATHOLOGIC CONDITIONS
OF THE UMBILICUS
Suprapubic dermoid sinuses usually extend from the skin overlying the pubis and pass over the superior surface of the bladder
to the umbilicus alongside the urachus.53,54 The embryologic
origin of such a sinus tract remains unclear, although it may
be a variant of a dorsal urethral duplication.
Children with bladder and cloacal exstrophy may have an
omphalocele or a low-set umbilicus incorporated into the
upper portion of the open bladder plate, along with diastasis
of the lower abdominal wall musculature and diastasis of
the symphysis pubis.55 Variants of exstrophy include superior
vesicointestinal fissure, duplicate exstrophy, and pseudoexstrophy, in which the bladder is intact and only the musculoskeletal
abnormalities are present.
Numerous unusual protrusions have been described at the
umbilicus. Ectopic pancreatic tissue including islets is best
explained by the pluripotential nature of cells of the vitelline
duct.56 Abnormal portions of liver connected to the main
lobes of the liver have been described and probably represent
entrapment by closure of the umbilical ring.57 A giant, 10-cm
hamartoma originating from the umbilicus without intraabdominal involvement has been excised without incident.58
The appendico-omphalic explanation of a fistula between the
appendix and the umbilicus was noted earlier (Omphalomesenteric Remnants). Entrapment of the appendix in the
umbilicus such as in a small omphalocele may also explain
some fistulas from the appendix to the umbilicus.59,60 Keloid
formation has been observed after umbilical cord separation.61
A giant umbilical cord may contain urachal remnants and
ectatic vessels and may mask a small omphalocele. Care should
be exercised during application of the cord clamp whenever the
appearance of the cord is abnormal.
The umbilicus may be affected by any disease of hairbearing skin including dermatoses and infections. It may be
the site of ectopic tissue including endometriosis, as well as
numerous primary and metastatic tumors, in addition to those
of urachal origin (see Table 74-2). Many acquired pathologic
conditions of the umbilicus are summarized in Table 74-3.
Umbilical Piercing
Umbilical piercing is common and may present dilemmas in
management. Trauma surgeons should be familiar with the
opening mechanisms of body piercings to facilitate radiology
studies and as needed for emergency procedures.62 Removal
of the piercing device is not necessarily recommended if
infection occurs.63 Local infections can be treated by warm
compresses and antibiotic ointment. If infection persists, oral

968

PART VII

ABDOMEN

TABLE 74-3
Acquired Conditions of the Umbilicus
Condition

Comment

Source

Dermatoses
Foreign body reactions
Omphalith
Pilonidal disease

Seborrheic dermatitis, psoriasis, herpes gestationis, Fabry disease

Starch, talc, inserted objects
Concretion of keratinous and sebaceous material
Related to hair-bearing sinus tracts

Infections
Endometriosis

Bacterial, fungal, viral, parasitic

Ectopic endometrial tissue

Benign tumors

Nevi, pyogenic granuloma, inclusion cysts, hemangioma, dermatofibroma, neurofibroma,

granular cell tumor, teratoma, desmoid tumor, lipoma
Melanoma, urachal adenocarcinoma, squamous cell carcinoma, basal cell carcinoma,
sarcoma, leiomyosarcoma

Powell, 1988a
Powell, 1988
Powell, 1988
Steck, 1965b
Sroujieh, 1989,c
Gupta, 1990d
Powell, 1988
Powell, 1988
Franklin, 1990e
Powell, 1988

Malignant tumors, primary

Malignant tumors, metastatic

Enteric fistulas

Psychiatric disorders
Miscellaneous disorders

Stomach, pancreas, endometrium, ovary, cervix, colon, small intestine, gallbladder, lung,
prostate, breast, unknown
Originate from Crohn disease, perforated appendicitis, other such visceral perforations
as colon, gallbladder
Symbolic vagina
Perforation from a ventriculoperitoneal shunt; infections, dermatoses, and granulation
tissue from piercing

Shetty, 1990f
Powell, 1988
Cornil, 1967g
Shetty, 1990
Park 1991h
Veloso, 1989i
Burchell, 1989j
Waltzer, 1974k
Bryant, 1988l
Lena 1994m

From Powell FC, Su WP: Dermatoses of the umbilicus. Int J Dermatol 1988;27:150-156.
Steck WD, Helwig EB: Umbilical granulomas, pilonidal disease and the urachus. Surg Gynecol Obstet 1965;120:1043-1057.
Sroujieh AS, Dawoud A: Umbilical sepsis. Br J Surg 1989;76:687-688.
d
Gupta S, Sikora S, Singh M, et al: Pilonidal disease of the umbilicusa report of two cases. Jpn J Surg 1990;20:590-592.
e
Franklin RR, Navarro C: Extragenital endometriosis. Prog Clin Biol Res 1990;323:289-295.
f
Shetty MR: Metastatic tumors of the umbilicus: A review 1830-1989. J Surg Oncol 1990;45:212-215.
g
Cornil C, Reynolds CT, Kickham CJ: Carcinoma of the urachus. J Urol 1967;98:93-95.
h
Park WH, Choi SO, Woo SK, et al: Appendicumbilical fistula as a sequela of perforated appendicitis. J Pediatr Surg 1991;26:1413-1415.
i
Veloso FT, Cardoso V, Fraga J, et al: Spontaneous umbilical fistula in Crohns disease. J Clin Gastroenterol 1989;11:197-200.
j
Burchell MC: Spontaneous umbilical fistula in Crohns disease. Report of a case. Dis Colon Rectum 1989;32:621-623.
k
Waltzer H: The umbilicus as vagina substitute. A clinical note. Psychoanal Q 1974;43:493-496.
l
Bryant MS, Bremer AM, Tepas JJ 3rd, et al: Abdominal complications of ventriculoperitoneal shunts. Case reports and review of the literature. Am Surg 1988;54:50-55.
m
Lena SM: Pierced navels are troublesome. CMAJ 1994;150:646-647.
b
c

antibiotics are prescribed. The site is cleansed with antibiotic

soap, and the jewelry rotated and left in place to allow drainage. Infections that require surgical drainage or debridement
are rare. Navel piercing jewelry may be temporarily removed
during surgery while preserving the piercing sinus tract. A
plastic intravenous catheter is placed in the sinus when the
piercing is removed.64

UMBILICAL LINT
The origin of umbilical lint has been a subject of curiosity and
speculation. Experimental shaving on the periumbilical hair
eliminates lint formation. Lint collected from the umbilicus
after colored cotton shirts were worn by subjects with intact
abdominal wall hair matched the color of the shirts indicating
the source of the lint. Presumably umbilical lint collects as
a direct result of the whorled umbilical hair acting on clothingderived material. Hair encircles the umbilicus, and the keratin
scales overlap with their bases pointing toward the hair follicle.
This arrangement imposes direction on the random movement
of the clothing lint that occurs when the material rubs back
and forth across the abdomen with body movement. The periumbilical hairs act in a ratchet-like fashion to move the lint into
the depths of the umbilicus.

Umbilical Hernia
------------------------------------------------------------------------------------------------------------------------------------------------

ANATOMY
At birth the umbilicus is surrounded by a dense fascial ring
that represents a defect in the linea alba. The umbilical opening is reinforced by strongly attached remnants of the umbilical arteries and urachus in an inferior direction and the more
weakly attached umbilical vein in a superior direction. A layer
of fascia (Richet fascia) derived from the transversalis fascia
supports the base of the umbilicus. The peritoneum forms
an intact undersurface of the umbilical ring, and skin overlies
the umbilicus after the cord has separated. When the supporting fascia of the umbilical defect is weak or absent, a direct
hernia results.65 An umbilical hernia in children is surrounded by the dense fascia of the umbilical ring, through
which a peritoneal sac attached to the overlying skin protrudes. The umbilical ring continues to close over time and
the fascia of the umbilical defect strengthens, which accounts
for the spontaneous resolution of this defect in most children.
An indirect umbilical hernia has also been described in
which the peritoneal contents herniate from a point immediately superior to the umbilical ring. The hernia follows the
umbilical canal along the umbilical vein, the linea alba in

CHAPTER 74

an anterior direction, and a thin layer of preperitoneal fascia

in a posterior direction.66 This form of herniation has been
suggested to cause proboscoid hernias in children; in this
defect, the umbilical cicatrix is displaced progressively in an
inferior direction as the hernia enlarges. This defect may also
be responsible for umbilical hernias in some adults.
The umbilical hernia of childhood is distinguished from
a hernia of the umbilical cord, in which there is a defect
in the peritoneum, as well as an open fascial defect at the
umbilicus. Intestines herniate into the substance of the umbilical cord itself and are covered only by amnion. A hernia of the
umbilical cord is, in effect, a small omphalocele.

INCIDENCE AND NATURAL HISTORY

There is no doubt a molecular basis for umbilical ring closure.67 Genetic heterogeneity accounts for the presence of
an open umbilical ring in some children at the time of birth,
whereas in others, the ring is essentially closed at the time of
cord separation. Unlike inguinal and epigastric hernias, which
have no real tendency to close after term, the umbilical ring is
programmed to continue closure in many children for weeks,
months, or years after birth.
Umbilical hernias in childhood occur with equal frequency
in boys and girls. Numerous reports document a high incidence in African and African American infants.6870 The umbilical ring is open throughout most of gestation but becomes
progressively smaller as gestation progresses. Most umbilical
hernias in infants are recognized after cord separation in the
first few weeks of life, and almost all are noted by 6 months
of age. Most undergo spontaneous closure during the first
3 years of life. Umbilical defects are found in many premature
infants after cord separation. Although umbilical hernias are
commonly found in low-birth-weight infants (75% of infants
weighing < 1500 g), most will resolve.71 The lack of accurate
longitudinal studies of children with umbilical defects does
not allow definitive conclusions to be drawn about their natural
history.72 Umbilical hernias with a small ring diameter (<1 cm)
are more likely to close spontaneously and close sooner than
those with a large ring diameter (>1.5 cm). The diameter of
the umbilical defect is prognostically important, whereas the
length of the protrusion is not. Some umbilical hernias that
are present at 5 years of age will close spontaneously without
an operation.73,74 The relationship between umbilical hernias
that become symptomatic later in life and childhood umbilical
defects is unknown. The protruding portion of the hernia generally remains unchanged while the fascial ring closes until it is
too small to admit any contents into the hernia sac. The hernia
thus tends to disappear abruptly.74 Umbilical hernias are commonly observed in patients with Down syndrome, trisomy 18,
trisomy 13, mucopolysaccharidoses, and congenital hypothyroidism. Umbilical defects (hernia or omphalocele) are part of
the Beckwith-Wiedemann syndrome. Incarceration of intestine
or omentum, strangulation, perforation, evisceration, and pain
are rare events in the natural history of umbilical hernias in
children. The most difficult task of the pediatric surgeon is to
convince the family that observation alone will be successful
in most cases and that an operation is not indicated for their
child, especially in infancy. The large conspicuous skin-covered
hernia sac with its characteristic and unsettling appearance is
often associated with a small fascial defect. It may be helpful
to demonstrate the size of the actual fascial defect to the parent.

DISORDERS OF THE UMBILICUS

969

Uncorrected umbilical hernias can become symptomatic

at any time in life. Rupture and evisceration are rare but
can occur.75,76 Incarceration is rare, but the small bowel is
most commonly affected when it does occur. Conditions that
increase intra-abdominal pressure increase the likelihood of
complications. Repair of umbilical hernias in patients with
ascites is hazardous. Umbilical hernias may also become
symptomatic during pregnancy, and if incarceration occurs,
surgery is required. Unusual contents of umbilical hernias
include uterine fibroids and endometrial elements.77,78

SURGICAL INDICATIONS
Although repair of childhood umbilical hernias has been
advocated to prevent the complication of incarceration in
adults, the relationship between the two events is unclear.79,80
Rare events such as incarceration requiring reduction, strangulation, perforation, and evisceration are absolute indications for
surgery. In the absence of these absolute indications, persistence
and appearance are relative indications for operative repair in
developed countries. Infants with giant proboscoid hernias in
whom the umbilical ring does not narrow during serial observations may be considered for repair in the first 2 years of life.
Typical umbilical hernias should be observed at least until age 2.
If there is no improvement in the size of the umbilical fascial
ring, consider repair. Ample evidence supports the decision
to postpone repair until later in childhood. Large defects
(>1.5 cm) that persist past the age of 5 should be repaired.
Evidence-based guidelines are lacking, and the decision may
be individualized on the basis of such considerations as family
history, parental desires, and local practices. The appearance of a
hernia often drives families to insist that the hernia be repaired.
In less developed parts of the world, it may be appropriate to
actively observe umbilical hernias, with operation reserved
for those with complications such as incarceration.81,82
If the child has a tender umbilical mass, the hernia may be
reduced by milking the air out of the incarcerated loop of
intestine and applying firm, steady pressure on the incarcerated
mass. Admitting a patient for observation to rule out peritonitis
and performing the operation the next day are appropriate. If
the incarceration resists reduction, an emergency procedure is
required. In an infant with an inguinal hernia and a concomitant
umbilical hernia, the umbilical hernia should generally be left
alone because it will probably close spontaneously.

SURGICAL TECHNIQUE, RESULTS,

AND COMPLICATIONS
Procedures described for the repair of umbilical hernias in
children range from multiple layers of closure after opening
the peritoneum to closed techniques in which the peritoneal
sac is inverted or treated like an inguinal hernia sac and ligated
with sutures.83,84 Absorbable and nonabsorbable sutures have
been advocated. The redundant skin of a large defect may be
left in place and improves in appearance over time. Some have
advocated excision of the skin and reconstruction when a
large proboscoid hernia is present (see later). However, the
fundamental technique of umbilical hernia repair has changed
little since the 1953 description by Gross.85 Secure closure of
the fascia, usually in a transverse fashion, and preservation of
the appearance of the umbilicus are common to all repairs.
Strapping and taping of the defects have been discredited.65,86

970

PART VII

ABDOMEN

Repair of an umbilical hernia is performed as an outpatient

procedure with the patient under general anesthesia. Local
anesthesia may be infiltrated into the wound before or after
the procedure, but paraumbilical infiltration avoids distortion of tissues by the anesthetic.87 Administration of local
anesthesia before the incision conforms to the principles of
preemptive analgesia. An infraumbilical skin crease incision
is made (Fig. 74-7). The incision may be hidden within the
umbilicus itself. Subcutaneous dissection is performed to
circumscribe the sac. The sac is transected and may be dissected from the undersurface of the umbilical skin, but extensive and time-consuming dissection is unnecessary. Leaving a
small remnant of the peritoneal sac on the undersurface of the
umbilical skin causes no complications. The sac may be
trimmed to a strong fascial edge or simply folded inward to
allow placement of interrupted absorbable sutures in a transverse orientation. To ensure accurate placement of sutures,
they are tied after placement is complete. A second layer of
closure is unnecessary. Inversion of the umbilical skin is maintained with fine absorbable dermal suture between the underside of the umbilicus and the midportion of the fascial closure.

The skin is closed with intradermal absorbable sutures and

covered with a small dressing. Some surgeons apply a pressure
dressing; however, a recent randomized study showed no
benefit from application of a pressure dressing in standard
childhood umbilical hernia repair.88 Although infection predisposes to recurrence of the hernia, such complications are
rare. Visceral injuries are possible but should not occur if
the fascial edges are kept in view during the procedure.
Epigastric hernias can occur immediately adjacent to the
umbilicus and may be difficult to distinguish from an umbilical hernia. Careful examination reveals a bulge at the upper
margin of the umbilicus or just above it. A supraumbilical incision permits repair of an adjacent epigastric hernia and simultaneous repair if both umbilical and epigastric hernias
are present.

Use of the Umbilicus

------------------------------------------------------------------------------------------------------------------------------------------------

Cannulation of the umbilical arteries and umbilical vein is

commonly performed in sick neonates and provides a convenient means for intravascular access and monitoring.

A
C

D
E
FIGURE 74-7 Repair of an umbilical hernia. A, An infraumbilical, curvilinear incision is marked. B, The sac is encircled and opened. C, The fascia is closed
transversely. D, A tacking suture is placed between the undersurface of the umbilical skin and the fascia. E, Final result.

CHAPTER 74

Associated risks are related to infection, vascular thrombosis,

and direct injury from catheters.89 There is insufficient
outcome-based evidence to either refute or support the use
of prophylactic antibiotics when umbilical artery or venous
catheters are inserted in newborns.90,91 There is little evidence that heparin coating of the catheter or catheter material
lowers complication rates.92 The umbilicus is frequently
used as the entry site for laparoscopic equipment. The center
of the umbilicus may be opened and the fascial ring probed
and spread for access in infants, small children, and those
with a shallow umbilicus. This technique leaves almost no
detectable incision. In patients with a deep umbilicus, a separate infraumbilical incision may be preferable. The umbilical
port is most often used as the primary site for placement of
the viewing camera. Closure of the fascial defect after removal
of the port is necessary to reduce the risk for formation of a
hernia. Single-port laparoscopic procedures using the umbilicus as the only access site are gaining popularity in both
pediatric and adult surgery.93 The umbilicus has also been
used to mask the abdominal incision used for pyloromyotomy
in cases of hypertrophic pyloric stenosis.94 This approach
results in an almost undetectable scar.
The umbilicus can also be used as a stoma site.95 No studies
have compared the complication rate for intestinal stomas
brought out of the umbilicus with those brought out from
other locations in the abdominal wall; however, complications
of umbilical stomas were reported by one study to be common.96 After closure, the umbilicus is reconstructed and a
nearly normal appearance is achieved. We have used the
umbilicus as a temporary ostomy site and have found it satisfactory. Though providing no physiologic benefit, it leaves the
patient with one less obvious incision site.
The umbilicus has also been used as an exit site for urinary
diversion. In premature infants, a temporary cutaneous vesicostomy brought out of the umbilicus functions well and can
be closed with excellent cosmetic results.97 Intestinal conduits for urinary diversion have also been brought out of the
umbilicus.98

Reconstruction and Preservation

of the Umbilicus
------------------------------------------------------------------------------------------------------------------------------------------------

The umbilicus is aesthetically and psychologically important,

and its abnormal appearance or absence may cause distress.
Absence of the umbilicus may even be a source of grief
and depression. The appearance of the umbilicus should be
acceptable to the patient and family. A T-shaped or oval umbilicus with a superior hood may be the most aesthetically
appealing configuration.3 A broad or protruding umbilicus
may be perceived to be less acceptable. One of the goals of
all umbilical surgical procedures is to maintain or restore as
normal an appearance as possible. Multiple techniques of
surgical reconstruction or re-creation of the umbilicus have
been described as noted later. Standard umbilical hernia
repair produces minimal distortion of the umbilicus and generally results in a satisfactory appearance. Omphalomesenteric
and urachal remnants can usually be excised through the
umbilicus as noted earlier. After laparoscopy via the

DISORDERS OF THE UMBILICUS

971

umbilicus, if the umbilical skin is secured to the fascial closure, a satisfactory umbilical depression is maintained. Patients
may seek surgical correction for the perception of an unfavorable appearance of the umbilicus such as protrusion. Umbilicoplasty to address the appearance of the umbilicus has become a
niche within the discipline of plastic and reconstructive surgery.

UMBILICOPLASTY FOR GIANT HERNIAS

WITH REDUNDANT SKIN
When large hernias (giant proboscoid hernias) are repaired,
the redundant skin results in an unnatural appearance. Some
improvement may occur with the passage of time, but a broad,
flat, protruding configuration may persist. Reconstruction by
a variety of techniques may improve the immediate and longterm appearance.99104 We have found the tripartite umbilicoplasty based on the technique described by Reyna and
colleagues to be satisfactory for immediate reconstruction of
giant umbilical hernias.103 None of these described techniques has been widely adopted and shown to be superior
in long-term follow-up.

UMBILICOPLASTY FOR ABDOMINAL

WALL DEFECTS AND CREATION
OF A NEO-UMBILICUS
The umbilicus may be retained or reconstructed during
the repair of abdominal wall abnormalities. The structures of the umbilical cord may be incorporated into a
reconstruction of the umbilicus, or a neo-umbilicus may
be fashioned.105111
In gastroschisis and omphalocele, fascial repair may be
performed through the circular skin defect that remains after
the umbilical structures have been excised. This is the case for
both primary and staged closures. The circular skin defect
may then be closed with an intradermal purse-string suture
that is incorporated into the middle of the fascial closure
(Fig. 74-8, A).106 Even if the fascial defect is enlarged for
the application of a Silastic chimney, the lower portion of
the defect can be closed in a similar circular fashion to create
the appearance of an umbilicus.105 Others have advocated
preservation of the umbilicus in the repair of abdominal wall
defects and leaving the umbilical remnants in place in continuity with the skin closure.108,110,111 There is a trend toward
preservation of the native umbilicus in gastroschisis repair
(Fig. 74-8, B).
The umbilicus is abnormally located in all children with
bladder exstrophy and is often associated with a small omphalocele defect. It may be transposed more cephalad at the time
of bladder closure to create a more normal appearance.112 In
children with prune-belly syndrome, the umbilicus may be
preserved on a vascularized pedicle and located appropriately
after the removal of excess skin.113
In some circumstances a new umbilicus must be constructed when it is absent as a result of previous surgical removal or
treatment of an abdominal wall defect. The normal location
for the umbilicus is at the level of the iliac crests, overlying
the third or fourth lumbar vertebrae. Umbilical reconstruction
should create a round or oval depression with steep walls that

972

PART VII

ABDOMEN

FIGURE 74-8 Technique of umbilicoplasty after excision of the cord structures or when a procedure is performed through the umbilicus (e.g., surgical
repair of gastroschisis or a small omphalocele, excision of an omphalomesenteric remnant through the umbilical ring, excision of a urachal remnant
through the umbilicus). A, Child with gastroschisis after fascial closure. Placement of a circumferential purse-string dermal suture is shown. Note that
the suture passes through the fascia. B, Retention of the umbilical cord after repair of gastroschisis.

is centrally fixed to the abdominal wall fascia (Fig. 74-9).

Some umbilical reconstructions tend to flatten over time.
Tubularized skin reconstructions may be more durable and
may also mimic the cushion or slightly raised area that surrounds the umbilical depression. Many techniques have been
proposed to reconstruct an absent umbilicus.106,109,114122
They vary considerably in their complexity, but none have
proved superior in long-term follow-up. Several techniques
have been specifically described to reconstruct an absent
umbilicus after exstrophy repair.112,123126
The complete reference list is available online at www.
expertconsult.com.

FIGURE 74-9 Creation of a neo-umbilicus using a tubularized defatted

skin pedicle in a patient with a giant omphalocele in conjunction with abdominal wall reconstruction.