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In an

autopsy series, Mitchell found closure of the processus vaginalis in


18% of full-term infants at birth (Mitchell, 1939). Among 1965
children undergoing unilateral inguinal hernia repair, Rowe identified
a patent contralateral processus vaginalis in 63% of patients
younger than 2 months and about 40% of those 1 to 2 years of age,
with similar frequency up until age 16 years (Rowe et al, 1969). The
incidence of incidental patency observed in older children and
adults at autopsy or laparoscopy is about 20% (Ajmani and Ajmani,
1983; van Wessem et al, 2003).
Definitions (Fig. 146-23)
Indirect inguinal hernia: a widely patent processus vaginalis extending
beyond the internal inguinal ring containing abdominal
contents (bowel, omentum, gonads) which may pass into the
inguinal canal, labia, or scrotum
Communicating hydrocele: a patent processus vaginalis extending
beyond the internal inguinal ring containing peritoneal fluid
alone, which extends to the testis, with fluid within the tunica
vaginalis
Hydrocele of the spermatic cord: fluid contained within a segment of
patent processus vaginalis with obliterated processus distally and
proximally
Scrotal hydrocele: fluid contained within the tunica vaginalis surrounding
the testis without communication proximally
Abdominoscrotal hydrocele: a large scrotal hydrocele that extends
proximally across the internal inguinal ring into the abdomen
without communication with the peritoneum
Epidemiology and Pathogenesis
Inguinal Hernia and Communicating Hydrocele
Inguinal hernias develop in 1% to 5% of children. The incidence is
5 to 10 times more common in boys and significantly more common
among premature infants (13% of babies born before 32 weeks and
nearly 30% of babies weighing less than 1 kg). The propensity for
the right side (3 : 1) is attributed to the later descent of the right
testicle. (Jones et al, 1998; Brandt, 2008). Female gender, prematurity,
age younger than 1 year, and history of cryptorchidism are
risk factors for bilaterality (Ein et al, 2006; Brandt, 2008). One
study suggests a protective effect of breastfeeding against the development
of inguinal hernias (Pisacane et al, 1995). Whereas hernias
may occur at any time during childhood, the average age at presentation
is 3 to 4 years, with nearly one third of cases manifesting
before age 6 months (Kapur et al, 1998). Concomitant hydroceles
are frequently seen; 19% of 6361 cases were found by Ein and colleagues
(2006) (70% scrotal, 26% cord, and 4% both) (Ein et al,
2006).
The majority of new hydroceles occurring after birth and
before puberty are associated with a patent processus vaginalis.
In an observational study, 59% of 302 newly identified hydroceles
in patients 1 to 18 (mean 4.4) years of age were clinically communicating
(clear history of fluctuation), and 6% were spermatic cord
hydroceles (Christensen et al, 2006). Seventy (65%) of the apparently
noncommunicating and 5 (29%) of the cord hydroceles were
followed, and spontaneous resolution was noted in 39 and 3, respectively.
Among boys undergoing hydrocele repair, complete obliteration
of the processus vaginalis was noted in 0% to 22% of cases
(Elder, 1992; Barthold and Redman, 1996; Han and Kang, 2002).
Noncommunicating Hydroceles
Hydroceles that spontaneously resolve during infancy or appear
during or after puberty are more commonly noncommunicating. These form
from delayed fluid absorption or abnormal fluid dynamics
within the tunica vaginalis or less commonly in response to
tumor, trauma, or inflammation. Simple scrotal hydroceles occur in
at least 5% of male neonates (Osifo and Osaigbovo, 2008) and are
typically bilateral, and resolve with fluid reabsorption.
Genetics and Associated Conditions
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Diagnosis
Signs and Symptoms
Inguinal hernias and communicating hydroceles typically manifest
as a painless bulge found in the groin or extending along the cord
to the scrotum. The bulge may be present only during periods of
increased intra-abdominal pressure (crying or bowel movements);
the supine position facilitates reduction of peritoneal fluid and
intra-abdominal contents. The presence of an intermittent bulge
helps to distinguish a reducible inguinal hernia and communicating
hydrocele from a scrotal hydrocele or hydrocele of the
spermatic cord. The child with an incarcerated inguinal hernia will
be irritable or inconsolable and have a persistent or larger bulge
without spontaneous reduction and may have decreased appetite
and signs of bowel obstruction (abdominal distention, vomiting,
and lack of flatus or stool).
The scrotal hydrocele may be seen as a chronic or acute scrotal
swelling after an inflammatory, infectious, or traumatic event. The
hydrocele size is typically stable but may decrease over time. The
hydrocele of the spermatic cord is also usually painless and variable
in size. It may be confused for the testis because of its round-oval
shape.

Physical Examination
Physical examination starts with a child standing, if age appropriate,
otherwise supine. Inspection proceeds from the lower abdominal
skin crease and along the inguinal canal to the scrotum. If the child
is crying, the bulge should be assessed to emerge or increase in size
and then improve or disappear when the child is consoled. Techniques
to increase intra-abdominal pressure may induce protrusion
of the bulge (immobilizing the extremities to induce crying in
infants, and jumping, coughing, laughing, blowing bubbles, or blowing up balloons in
older children) (Brandt, 2008). If a bulge
is not elicited at the time of examination, photographs of the
bulge taken by family members are diagnostically reliable (Kawaguchi
and Shaul, 2009).
Palpation proceeds craniocaudally from superior-lateral to the
pubic tubercle down to the scrotum to determine the proximal and
distal extent of the swelling. Communicating hydroceles and hernias
start at the level of the internal ring and end variably. The silkstocking
sign (sensation of rubbing silk together), sought by rubbing
the cord structures side to side near the pubic tubercle, implies
thicker cord structures. A hydrocele of the spermatic cord may be
confused with a testis, but normal cord structures are palpable
above and below and a testis will also be palpated. Scrotal hydroceles
may elicit a blue hue through the scrotal skin. Normal cord
structures are palpable superior to the hydrocele but may be difficult
to distinguish if it extends up to or across (abdominoscrotal) the
internal ring, in which case abdominal examination should reveal
a ballotable mass. The hydrocele fluid surrounding the testicle
should transilluminate; however, neonatal bowel may also transilluminate.
The testis should be palpable within a soft scrotal hydrocele
but may be difficult to discern within a tense hydrocele.

Radiologic Imaging
Imaging is often of limited usefulness. Ultrasonography may identify
a large elongated echolucent area from the groin extending
anteromedially in the spermatic cord; omentum or bowel with
peristalsis can be found in a large hernia sac (Fig. 146-24). In
the presence of a presumed hydrocele, a sonogram can aid in
identifying an unpalpable testicle surrounded by hydrocele fluid
(Fig. 146-25).
Surgical Repair
Inguinal Hernia
Inguinal hernias require surgical repair shortly after diagnosis,
given the significant risk of associated complications. Outpatient
surgery can be performed within a few weeks in easily reducible
hernias or communicating hydroceles and more urgently if there
is moderate difficulty in reducing the hernia contents. Parental
counseling regarding the signs and symptoms of incarceration
should occur. An irreducible hernia requires immediate exploration.
Hernias in premature infants can be repaired before hospital

A genetic predisposition to developing inguinal hernias exists,


and potential genome sites have been identified. Among probands,
there is a 28% risk in other family members and a higher relative
risk in first-degree relatives (6.9), particularly female siblings (17.8)
of affected girls (Gong et al, 1994; Jones et al, 1998). Genetic transmission
appears to be autosomal dominant with reduced penetrance.
Zheng has implicated DNA sequence variants (DSVs) within
T-box transcription factor 1 (TBX1) and TBX2 as rare causes (Zhang
et al, 2014a, 2014b). Sezer identified polymorphisms of the collagen
type I 1 (COL1A1). The Sp1 binding site was associated with
a higher risk of developing inguinal hernias (Sezer et al, 2014).
Hernias are associated with other diseases and anomalies. These
include bladder exstrophy, epididymal anomalies, connective tissue
disorders, cystic fibrosis, and posterior urethral valves. Inguinal
hernia is also a component of over 200 syndromes, many of which
have known specific genetic alterations (Winter-Baraitser Dysmorphology
Database, www.lmdatabases.com). Other genital anomalies
such as polyorchidism, transverse testicular ectopia with or
without persistent mllerian ducts, and splenogonadal fusion may
be seen at the time of hernia repair. Abnormal epididymal-testicular
attachment correlates with degree of closure of the processus vaginalis
(Elder, 1992; Barthold and Redman, 1996). Finally, the presence
of abdominal ascites or a ventriculoperitoneal shunt increase
the chances of developing an inguinal hernia (Grosfeld et al, 1991).

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