recommended, and the importance of preservation of the

appearance of the umbilicus rather than excision was empha-

sized. 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. Cullen’s curiosity was
originally stimulated by a case of cancer at the umbilicus, and
it inspired him to explore the entire topic of umbilical pathol-
ogy. 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 struc-
tures 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 sur-
geons 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 com-
monly performed operations in childhood. In addition, the
CHAPTER 74 umbilicus serves as a portal of entry for most laparoscopic pro-
cedures, 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.
Disorders 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
of the Umbilicus piercings to enhance its appearance.

Robert E. Cilley Normal Embryology

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

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 trilami-
History nar embryo that begins in the fourth gestational week. How-
------------------------------------------------------------------------------------------------------------------------------------------------
ever, the actual growth of the embryo does not truly involve
Umbilical malformations have been depicted in art and sculp- “bending” and “folding” of structures but rather represents
ture since antiquity, but the developmental basis for these differential growth of tissues. Initially, the amnion is located
abnormalities was not recognized until the late nineteenth in a dorsal direction, whereas the yolk sac occupies a ventral
century. Surgical textbooks, such as that by von Bergmann position. The embryo is attached to the chorion, the forerun-
in 1904, clearly describe the embryology responsible for per- ner of the placenta, by a connecting stalk composed of extra-
sistence of the vitellointestinal duct as a fistula, sinus, or cyst.1 embryonic mesoderm in which the umbilical vessels develop
The symptoms of fecal drainage (“congenital umbilical anus”) and into which the allantois grows (Fig. 74-1, A). The yolk sac
and prolapse of the intestine were well known. The surgeon maintains its ventral position but is divided into intracoelomic
was advised to avoid pitfalls such as excision of an “umbilical and extracoelomic portions (Fig. 74-1, B). The intracoelomic
tumor” that exposed two intestinal lumens because it would portion, derived from the roof of the yolk sac, becomes the
indicate that the vitellointestinal remnant had been excised primitive alimentary canal and maintains a connection with
in excess back to the ileum. An umbilical polyp represen- the extracoelomic portion through the vitelline or omphalo-
ting a persistent remnant of the duct was referred to as an mesenteric duct. This connection is normally lost by the fifth
“enteroteratoma.” to seventh week of gestation.2,4 Persistence of this connection,
Surgical management has changed little in the past 100 years. as a remnant of either the developing alimentary tract or the
Interestingly, then as now, granulomas of the umbilical cord accompanying vitelline vessels, accounts for some of the
were treated by silver nitrate cauterization. The embryologic abnormalities described in this chapter.
basis of developmental abnormalities of the urachus was sim- Early in the third week of gestation, a diverticulum called
ilarly recognized, and their surgical treatment was described the allantois forms from the posterior wall of the yolk sac
much as it is today. The natural history of spontaneous resolu- and extends into the connecting stalk of the embryo
tion of most umbilical hernias was also understood at the end (Fig. 74-1, A and B). The allantois serves as a reservoir for
of the nineteenth century. External compression was often the developing renal system in lower vertebrates but has no
961

booksmedicos.org
962 PART VII ABDOMEN

A B

C D
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.

booksmedicos.org
 CHAPTER 74 DISORDERS OF THE UMBILICUS 963

known role in human development; it remains rudimentary as cannot be guaranteed. These agents may affect cord separation
the transitory extraembryonic portion of the urachus. As the time, some cause discoloration, and repeated use of iodine-
distal hindgut, or cloaca, partitions into the urogenital sinus containing antimicrobials may result in systemic absorption
ventrally and the anorectal canal dorsally, the developing blad- of iodine and suppression of thyroid function. There is good
der remains connected by the urachus to the allantois within evidence that, in developed countries, dry cord care, without
the body stalk (see Fig. 74-1, D). The urachus is derived the application of topical antibiotics, in association with rou-
wholly from the developing bladder and may persist in various tine soap and water bathing and meticulous hand washing
forms, which accounts for the abnormalities described later. practices is as effective as topical agents in reducing infection.7
As the embryo develops, the amnion is drawn around it to However, a recent randomized prospective study demon-
surround the embryo and cover all the developing umbilical strated fewer cord-related complications in infants treated
cord structures including the allantois, umbilical vessels, vitel- with chlorhexidine powder compared with standard dry
line duct, and primitive mesenchymal tissue (Wharton jelly) cord care.8 In the undeveloped world, antiseptic cord cleans-
(Fig. 74-1, C). During the period of rapid intestinal growth ing with chlorhexidine may significantly reduce neonatal
between the sixth and tenth weeks of gestation, the develop- morbidity and mortality.9
ing midgut is extracoelomic. As the body wall continues to Intestinal injury may result from injudicious placement of an
develop, the intestines are incorporated into the coelomic umbilical cord clamp when an unrecognized small hernia of the
cavity and intestinal rotation and fixation progress. The fibro- umbilical cord (i.e., a small omphalocele) is present. Abdominal
muscular umbilical ring continues to contract and is nearly wall defects that relate to the umbilicus (i.e., gastroschisis and
closed by the time of birth (Fig. 74-1, D). Persistence of the omphalocele) are covered in Chapter 75.
fascial opening as an umbilical hernia occurs frequently and The normal time for separation of the umbilical cord after
is commonly seen in premature infants. Unlike other abdom- birth ranges from 3 days to 2 months.10 Antimicrobial treat-
inal wall defects, umbilical hernias tend to resolve without ment may prolong cord separation by decreasing leukocyte
specific treatment as a result of the ongoing development of infiltration. Delayed separation of the umbilical cord has been
tissues at the umbilical ring after birth. The fate of the struc- associated with heritable neutrophil mobility defects and
tures that relate to the development of the umbilicus is shown widespread infections that are often lethal.11 The abnormal
in Table 74-1. neutrophils lack a membrane glycoprotein, which results
in abnormal attachment, chemotaxis, and phagocytosis.10
Although persistence of umbilical cord attachment beyond
Umbilicus at Birth 3 weeks of age has been suggested to be a sign of such immu-
------------------------------------------------------------------------------------------------------------------------------------------------
nologic abnormalities, recent studies that have included more
Modern obstetric practice uses plastic clamps that are placed a than 600 newborns have demonstrated the range of normal
few centimeters from the umbilical skin during cord division newborn cord separation to be broad (3 to 67 days), with a
at the time of delivery. Topical antimicrobials such as triple mean of 14 to 15 days.10,12 In these studies, nearly 10% of
dye, bacitracin, silver sulfadiazine, povidone-iodine, chlor- normal newborns underwent cord separation after 3 weeks
hexidine, hexachlorophene, alcohol, salicylic sugar powder, of age, thus indicating that delayed cord separation is not a
green clay powder, silver-benzyl-peroxide powder, and 1% reliable indicator of immunologic disease. If prolonged cord
basic fuchsin may be applied to the cord after birth.5,6 All separation is associated with umbilical infection, leukocyte
these agents are effective in reducing bacterial colonization adhesion deficiency disorders should be suspected and an
rates, and their use is recommended when adequate cord care 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 Obliterates Median umbilical ligament, patent urachus,
to the allantois) sinus, cyst
Omphalomesenteric duct Obliterates Meckel diverticulum, patent omphalomesenteric duct,
(connects the midgut to the sinus, cyst, bands, polyp
yolk sac)
Omphalomesenteric arteries Most regress; fuse to form the celiac, superior mesenteric, Dominant artery may accompany Meckel diverticulum,
and inferior umbilicus fibrous band to the mesenteric arteries
Omphalomesenteric veins Plexus around the duodenum becomes the superior Preduodenal portal vein if the ventral portion
mesenteric and portal vein (contribution from both of the plexus persists
the left and right vein)
Umbilical arteries Obliterate after birth Medial or lateral umbilical ligaments*
Umbilical veins Right obliterates; left returns placental blood to Falciform ligament
the inferior vena cava through the ductus venosus

*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.

booksmedicos.org
964 PART VII ABDOMEN

After separation of the cord, the umbilicus may have many Excision of preperitoneal tissue including the umbilical vessels
appearances. A normal umbilicus is characterized by a depres- and urachal remnant may be critically important to achieve
sion in which may be found the mamelon (a central eminence eradication of the infection because these tissues harbor invasive
that contains the remnants of the solid portion of the umbilical bacteria and may provide a route for the progressive spread of
cord) and the cicatrix (dense scar where the intraembryonic and infection seen after less extensive surgical debridement.22 The
extraembryonic coelom were in continuity). The cushion is the defect may require a temporary prosthetic patch for closure,
slightly raised margin that surrounds the umbilical depression. but ultimate fascial closure and umbilical reconstruction may
Cullen described more than 60 “normal” configurations of the leave an acceptable appearance. Hyperbaric oxygen therapy
umbilicus.2 has been advocated as adjuvant therapy, but it is not of pro-
ven benefit.26 The overall reported mortality associated with
necrotizing fasciitis in collected series is 81%.18,22–27
Umbilical Abnormalities Umbilical drainage resulting from chronic infection of
------------------------------------------------------------------------------------------------------------------------------------------------
umbilical remnants such as umbilical artery remnants has also
ACQUIRED been reported.28 Excision and debridement are curative.
Omphalitis can result in necrosis and breakdown of the
Umbilical Granuloma umbilical stump with spontaneous evisceration within the
After cord separation, a small mass of granulation tissue may first 2 months of life and may be associated with portal venous
develop at the base. These granulomas consist of true granu- thrombosis and subsequent extrahepatic portal hypertension.
lation tissue with fibroblasts and abundant capillaries; the
granulomas range in size from 1 mm to approximately 1 cm. CONGENITAL
The surface often has a pedunculated appearance. Umbilical
Omphalomesenteric Remnants
granulomas may be treated by cauterization with one or more
applications of silver nitrate until the area epithelializes. Alter- Remnants of the vitelline or omphalomesenteric duct account
natively, the granuloma may be excised and silver nitrate or for a wide variety of umbilical abnormalities that may require
absorbable hemostatic material applied.15 If the mass does surgical correction.28a These remnants include fistulas, sinus
not respond to cauterization, a true umbilical polyp or sinus tracts, cysts, mucosal remnants, and congenital bands. Typi-
tract must be suspected (see later). Care must be taken with cal variations of the pathologic varieties are illustrated in
silver nitrate application because burns and skin injury may Figure 74-2, A to F.2,29,30
occur.16 If the omphalomesenteric duct is patent from the terminal
ileum to the umbilicus, fecal umbilical drainage will be noted
Umbilical Infections (Fig. 74-3, A). Although this event is dramatic to parents, the
Although modern perinatal practice has dramatically reduced problem is immediately recognizable on examination and
the incidence of omphalitis, infections of the umbilicus still parents may be reassured that prompt surgical correction is
occur with alarming morbidity and mortality, particularly in curative. Prolapse of the proximal and distal ileum through
undeveloped countries.17 Rigorous asepsis, hand washing, the patent duct has a characteristic appearance. Although con-
and cord care (either dry cord care or topical antimicrobials) trast injections are of interest, they do not change the surgical
have reduced the incidence of umbilical infections to less than approach (Fig. 74-3, B). Anatomically unusual conditions
1% in hospitalized newborns.18 Before the institution of such as an unexpected origin of the omphalomesenteric duct
such practices, the mortality rate for omphalitis was 65%. from the appendix will be recognized at the time of operation
The primary pathogens implicated in these infections were (Fig. 74-4).31,32 Unless another, more serious medical con-
Staphylococcus aureus and Streptococcus pyogenes. Currently, dition exists, a patent omphalomesenteric duct should be
gram-negative bacteria play an important role in the patho- excised promptly. A mechanical intestinal preparation is not
genesis of umbilical infections. Severe infections are often necessary, although we customarily stop formula feeding;
polymicrobial. Omphalitis may be manifested as a purulent perioperative intravenous antibiotics are also given. The oper-
umbilical discharge or periumbilical cellulitis. Delivery at ation may be performed through the umbilicus itself or
home, low birth weight, use of umbilical catheters, and septic through an incision below the umbilicus. Full exploration
delivery are risk factors. Tetanus infection occurs on rare and identification of all umbilical structures including one
occasions. Intravenous antibiotic therapy is effective in erad- vein, two arteries, and the urachal remnant are indicated.
icating most infections. Omphalitis is a common problem in The omphalomesenteric duct is traced to the ileum and
developing countries, where it accounts for more than a divided. The ileum is closed, and care must be taken to control
quarter of neonatal hospital admissions.19,20 any dominant vitelline vessels that may be present. After the
Cellulitis may progress to fasciitis, and such progression fascia is closed, umbilicoplasty is performed.
may be subtle. Signs of necrotizing fasciitis include abdominal Small duct remnants and sinuses may have less characteris-
distention, tachycardia, purpura, blistering, pyrexia, hypo- tic drainage. Injection of contrast material may be helpful in
thermia, leukocytosis, and progression of cellulitis despite delineating the nature of the problem in these instances, but
antibiotic therapy. Bacteriologic cultures demonstrate polymi- surgical exploration remains the definitive diagnostic test. It
crobial flora.21 Necrotizing fasciitis and umbilical gangrene is important that a full exploration is performed and that all
may be lethal and require immediate wide surgical debride- umbilical structures including the intraperitoneal undersurface
ment for patient survival.18,22–27 Excision should be perfor- of the umbilicus are visualized to identify and remove any
med immediately on recognition; all infected skin, fat, and bands attached to the small intestine. If a Meckel diverticulum
fascia should be excised back to viable, bleeding abdomi- is attached to an omphalomesenteric band discovered at explo-
nal wall musculature. The umbilicus is obligatorily excised. ration, it is excised. Cystic remnants of the omphalomesenteric

booksmedicos.org
 CHAPTER 74 DISORDERS OF THE UMBILICUS 965

A B C

D E F
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.

A B
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 sur-
gical 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 omphalo-
mesenteric duct may occur.33,34 In one case the defect was

booksmedicos.org
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 regres-
sion 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 umbi-
licus. 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]: Swenson’s 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

A B C
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.

booksmedicos.org

the umbilicus and the suprapubic area. Ultrasonography or
computed tomography may be helpful to confirm the diagno-
sis. Other unusual manifestations have been reported includ-
ing a lateral mass.40 The urachus has also been described as
exiting from the midline below the umbilicus.41,42 In addi-
tion, a patent urachus may be one of the causes of a giant um-
bilical 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. Ab-
normal 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 includ-
ing 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 injec-
tions, computed tomography, and magnetic resonance imaging
may occasionally be helpful in diagnosing and treating umbil-
ical 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 fea-
tures may have characteristic findings that aid in diagnosis.
Minor abnormalities that lack medical significance can none-
theless provide insight into the nature and timing of dysmor-
phologic events that occur during development.51 Commonly,
dermatoglyphics, hair patterning, auricular shape, and genital
configuration are part of such observations. Minor abnormal-
ities 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)
booksmedicos.org
CHAPTER 74 DISORDERS OF THE UMBILICUS 967
and genital hypoplasia. If the umbilicus is broad and promi-
nent with a large stalk and redundant periumbilical skin,
Rieger syndrome should be suspected, especially if these
umbilical abnormalities occur in conjunction with goniodys-
genesis and hypodontia. If the umbilicus is prominent with
a button-like central portion in a deep longitudinally orien-
ted ovoid depression or flat with radiating branches of the
cicatrix, Aarskog syndrome, a condition classically character-
ized 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 over-
lying 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 pseudoexstro-
phy, 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 intra-
abdominal involvement has been excised without incident.58
The appendico-omphalic explanation of a fistula between the
appendix and the umbilicus was noted earlier (“Omphalome-
senteric 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 hair-
bearing 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 Seborrheic dermatitis, psoriasis, herpes gestationis, Fabry disease Powell, 1988a
Foreign body reactions Starch, talc, inserted objects Powell, 1988
Omphalith Concretion of keratinous and sebaceous material Powell, 1988
Pilonidal disease Related to hair-bearing sinus tracts Steck, 1965b
Sroujieh, 1989,c
Gupta, 1990d
Infections Bacterial, fungal, viral, parasitic Powell, 1988
Endometriosis Ectopic endometrial tissue Powell, 1988
Franklin, 1990e
Benign tumors Nevi, pyogenic granuloma, inclusion cysts, hemangioma, dermatofibroma, neurofibroma, Powell, 1988
granular cell tumor, teratoma, desmoid tumor, lipoma
Malignant tumors, primary Melanoma, urachal adenocarcinoma, squamous cell carcinoma, basal cell carcinoma, Shetty, 1990f
sarcoma, leiomyosarcoma Powell, 1988
Cornil, 1967g
Malignant tumors, metastatic Stomach, pancreas, endometrium, ovary, cervix, colon, small intestine, gallbladder, lung, Shetty, 1990
prostate, breast, unknown
Enteric fistulas Originate from Crohn disease, perforated appendicitis, other such visceral perforations Park 1991h
as colon, gallbladder Veloso, 1989i
Burchell, 1989j
Psychiatric disorders Symbolic vagina Waltzer, 1974k
Miscellaneous disorders Perforation from a ventriculoperitoneal shunt; infections, dermatoses, and granulation Bryant, 1988l
tissue from piercing Lena 1994m
a
From Powell FC, Su WP: Dermatoses of the umbilicus. Int J Dermatol 1988;27:150-156.
b
Steck WD, Helwig EB: Umbilical granulomas, pilonidal disease and the urachus. Surg Gynecol Obstet 1965;120:1043-1057.
c
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 umbilicus—a 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 Crohn’s disease. J Clin Gastroenterol 1989;11:197-200.
j
Burchell MC: Spontaneous umbilical fistula in Crohn’s 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.

antibiotics are prescribed. The site is cleansed with antibiotic

soap, and the jewelry rotated and left in place to allow drain- Umbilical Hernia
------------------------------------------------------------------------------------------------------------------------------------------------

age. 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 clothing-
derived 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 perium-
bilical hairs act in a ratchet-like fashion to move the lint into
the depths of the umbilicus.
ANATOMY
At birth the umbilicus is surrounded by a dense fascial ring
that represents a defect in the linea alba. The umbilical open-
ing is reinforced by strongly attached remnants of the umbil-
ical 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 support-
ing fascia of the umbilical defect is weak or absent, a direct
hernia results.65 An umbilical hernia in children is sur-
rounded by the dense fascia of the umbilical ring, through
which a peritoneal sac attached to the overlying skin pro-
trudes. 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 immedi-
ately superior to the umbilical ring. The hernia follows the
umbilical canal along the umbilical vein, the linea alba in

booksmedicos.org

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 umbil-
ical 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 clo-
sure.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 inci-
dence in African and African American infants.68–70 The umbil-
ical 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 gen-
erally 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 com-
monly observed in patients with Down syndrome, trisomy 18,
trisomy 13, mucopolysaccharidoses, and congenital hypothy-
roidism. 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.
booksmedicos.org
CHAPTER 74 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, strangu-
lation, 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 obser-
vations 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 distor-
tion 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 dis-
sected from the undersurface of the umbilical skin, but exten-
sive 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 trans-
verse 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 main-
tained with fine absorbable dermal suture between the under-
side of the umbilicus and the midportion of the fascial closure.
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.
booksmedicos.org
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 pre-
disposes 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 umbil-
ical hernia. Careful examination reveals a bulge at the upper
margin of the umbilicus or just above it. A supraumbilical in-
cision permits repair of an adjacent epigastric hernia and si-
multaneous 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 con-
venient means for intravascular access and monitoring.
 CHAPTER 74 DISORDERS OF THE UMBILICUS 971

Associated risks are related to infection, vascular thrombosis, umbilicus, if the umbilical skin is secured to the fascial clo-
and direct injury from catheters.89 There is insufficient sure, a satisfactory umbilical depression is maintained. Patients
outcome-based evidence to either refute or support the use may seek surgical correction for the perception of an unfavor-
of prophylactic antibiotics when umbilical artery or venous able appearance of the umbilicus such as protrusion. Umbilico-
catheters are inserted in newborns.90,91 There is little evi- plasty to address the appearance of the umbilicus has become a
dence that heparin coating of the catheter or catheter material niche within the discipline of plastic and reconstructive surgery.
lowers complication rates.92 The umbilicus is frequently
used as the entry site for laparoscopic equipment. The center UMBILICOPLASTY FOR GIANT HERNIAS
of the umbilicus may be opened and the fascial ring probed WITH REDUNDANT SKIN
and spread for access in infants, small children, and those
with a shallow umbilicus. This technique leaves almost no When large hernias (giant proboscoid hernias) are repaired,
detectable incision. In patients with a deep umbilicus, a sep- the redundant skin results in an unnatural appearance. Some
arate infraumbilical incision may be preferable. The umbilical improvement may occur with the passage of time, but a broad,
port is most often used as the primary site for placement of flat, protruding configuration may persist. Reconstruction by
the viewing camera. Closure of the fascial defect after removal a variety of techniques may improve the immediate and long-
of the port is necessary to reduce the risk for formation of a term appearance.99–104 We have found the “tripartite umbili-
hernia. Single-port laparoscopic procedures using the umbili- coplasty” based on the technique described by Reyna and
cus as the only access site are gaining popularity in both colleagues to be satisfactory for immediate reconstruction of
pediatric and adult surgery.93 The umbilicus has also been giant umbilical hernias.103 None of these described tech-
used to mask the abdominal incision used for pyloromyotomy niques has been widely adopted and shown to be superior
in cases of hypertrophic pyloric stenosis.94 This approach in long-term follow-up.
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 UMBILICOPLASTY FOR ABDOMINAL
brought out of the umbilicus with those brought out from WALL DEFECTS AND CREATION
other locations in the abdominal wall; however, complications
OF A NEO-UMBILICUS
of umbilical stomas were reported by one study to be com-
mon.96 After closure, the umbilicus is reconstructed and a The umbilicus may be retained or reconstructed during
nearly normal appearance is achieved. We have used the the repair of abdominal wall abnormalities. The struc-
umbilicus as a temporary ostomy site and have found it satis- tures of the umbilical cord may be incorporated into a
factory. Though providing no physiologic benefit, it leaves the reconstruction of the umbilicus, or a neo-umbilicus may
patient with one less obvious incision site. be fashioned.105–111
The umbilicus has also been used as an exit site for urinary In gastroschisis and omphalocele, fascial repair may be
diversion. In premature infants, a temporary cutaneous vesi- performed through the circular skin defect that remains after
costomy brought out of the umbilicus functions well and can the umbilical structures have been excised. This is the case for
be closed with excellent cosmetic results.97 Intestinal con- both primary and staged closures. The circular skin defect
duits for urinary diversion have also been brought out of the may then be closed with an intradermal purse-string suture
umbilicus.98 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
Reconstruction and Preservation the defect can be closed in a similar circular fashion to create
the appearance of an umbilicus.105 Others have advocated
of the Umbilicus preservation of the umbilicus in the repair of abdominal wall
------------------------------------------------------------------------------------------------------------------------------------------------
defects and leaving the umbilical remnants in place in conti-
The umbilicus is aesthetically and psychologically important, nuity with the skin closure.108,110,111 There is a trend toward
and its abnormal appearance or absence may cause distress. preservation of the native umbilicus in gastroschisis repair
Absence of the umbilicus may even be a source of grief (Fig. 74-8, B).
and depression. The appearance of the umbilicus should be The umbilicus is abnormally located in all children with
acceptable to the patient and family. A T-shaped or oval umbi- bladder exstrophy and is often associated with a small ompha-
licus with a superior hood may be the most aesthetically locele defect. It may be transposed more cephalad at the time
appealing configuration.3 A broad or protruding umbilicus of bladder closure to create a more normal appearance.112 In
may be perceived to be less acceptable. One of the goals of children with prune-belly syndrome, the umbilicus may be
all umbilical surgical procedures is to maintain or restore as preserved on a vascularized pedicle and located appropriately
normal an appearance as possible. Multiple techniques of after the removal of excess skin.113
surgical reconstruction or re-creation of the umbilicus have In some circumstances a new umbilicus must be construc-
been described as noted later. Standard umbilical hernia ted when it is absent as a result of previous surgical removal or
repair produces minimal distortion of the umbilicus and gen- treatment of an abdominal wall defect. The normal location
erally results in a satisfactory appearance. Omphalomesenteric for the umbilicus is at the level of the iliac crests, overlying
and urachal remnants can usually be excised through the the third or fourth lumbar vertebrae. Umbilical reconstruction
umbilicus as noted earlier. After laparoscopy via the should create a round or oval depression with steep walls that

booksmedicos.org
972 PART VII ABDOMEN

A B
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 sur-
rounds the umbilical depression. Many techniques have been
proposed to reconstruct an absent umbilicus.106,109,114–122
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.112,123–126

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 ab-
dominal wall reconstruction.

booksmedicos.org