Hernias are among the most common conditions encountered

Chapter 69
Uncommon Hernias
Shaheen J. Timmapuri and Rajeev Prasad
Hernias are among the most common conditions encountered
by pediatric surgeons. Although inguinal and umbilical hernias are by far the most prevalent, rarer types of hernias are
occasionally encountered in children. Because they are so
rare, most of the information regarding the treatment of these
conditions is anecdotal. A clear understanding of the anatomy and techniques for repair of these hernias is critical for
developing an appropriate management plan. An incorrect or
delayed diagnosis can lead to significant complications,
including bowel obstruction, ischemia, or perforation.
Unusual Inguinal Hernias
Incarcerated hernias with unusual contents and historically
named after Littre, Amyand, and Richter, can occur in any
abdominal wall or inguinal defect. Although rare in any age
group, Littre’s hernia, in which the hernia sac contains a
Meckel’s diverticulum, are extremely uncommon in the pediatric population. Preoperative diagnosis is nearly impossible.
Radiographic evaluation (US, CT) is often unhelpful, aside
from confirming the presence of a hernia. The usual evaluation for a suspected incarcerated hernia should be undertaken
to ensure prompt reduction and to prevent ischemic consequences. Besides bowel ischemia, other complications include
pain, bleeding, and perforation. Inflammation of the Meckel’s
diverticulum can create dense adhesions to the hernia sac. The
optimal treatment consists of hernia repair and resection of the
diverticulum. If thorough inspection and proper resection are
not possible through the inguinal incision, it might be necessary to make an additional incision on the abdomen. Proponents
of laparoscopic hernia repair have reported cases in which
the Meckel’s diverticulum was identified and reduced laparoscopically, and then resected through an umbilical incision.
This approach might allow better visualization of the bowel
than is possible through a standard inguinal incision.
Amyand’s hernia is an inguinal hernia in which the hernia
sac contains a normal or acutely inflamed appendix. As with
Littre’s hernias, they are nearly impossible to diagnose preoperatively. The presentation is similar to that of any incarcerated or strangulated inguinal hernia: tenderness, erythema,
inability to reduce the hernia contents. There are several case
reports in the literature of acute appendicitis occurring within
a hernia sac mimicking a strangulated inguinal hernia or testicular torsion. The significance of acute appendicitis within
the sac is unclear. Inflammation of the appendix can be incidental or perhaps a consequence of incarceration.
The treatment of Amyand’s hernias consists of hernia repair
and appendectomy, which can be performed using a laparoscopic-assisted approach. Postoperative management depends
on the condition of the appendix at the time of surgery.
Prolonged hospitalization for long-term antibiotics is sometimes necessary for perforated appendicitis and patients should
be monitored for complications such as intra-abdominal
abscess and sepsis.
Richter’s hernia occurs when only the antimesenteric portion of a segment of bowel wall protrudes through a hernia
defect. Because the entire circumference of the intestine is not
involved, these hernias do not present with the usual symptoms
of a bowel obstruction even in the setting of incarceration. This
can lead to bowel wall ischemia and perforation if unrecognized or if an involved ischemic segment is unknowingly
reduced. Therefore, surgeons should have a high index of suspicion for ischemia if a child has abdominal symptoms following hernia reduction or repair. The treatment is a standard
hernia repair, with careful inspection of the involved intestinal
segment, if necessary using an abdominal counter-incision.
Femoral Hernias
S.J. Timmapuri (*)
Department of Pediatric Surgery, Drexel University, St. Christopher’s
Hospital for Children, Erie Ave at Front Street, Philadelphia,
PA 19134, USA
e-mail: [email protected]
Femoral hernias account for approximately 1% of pediatric
groin hernias. They are frequently misdiagnosed, as often as
75% in some series. The femoral hernia is often confused
P. Mattei (ed.), Fundamentals of Pediatric Surgery,
DOI 10.1007/978-1-4419-6643-8_69, © Springer Science+Business Media, LLC 2011
with an indirect inguinal hernia and the correct diagnosis is
usually made upon inguinal exploration. It can also be mistaken for an enlarged lymph node, lipoma, or abscess.
Because these hernias are so rare in children, understanding
the anatomy is critical for making a correct diagnosis and
ensuring appropriate and prompt treatment.
The etiology remains unclear but an anatomic defect, such
as an enlarged femoral ring, is probably the cause in most cases.
Though more common in adults, acquired etiologies (increased
intra-abdominal pressure, prior inguinal surgery) are in some
cases thought to be contributing factors. The majority of pediatric patients with femoral hernias are less than 10 years of age,
supporting a congenital rather than acquired etiology.
As the diagnosis is usually not considered preoperatively,
many patients present with a recurrent groin bulge after an
inguinal hernia repair. Femoral hernias are located lateral
and inferior to the pubic tubercle and inferior to the inguinal
ligament. The diagnosis can usually be made by thorough
physical examination. Some patients complain of pain in the
ipsilateral lower extremity, which is caused by pressure on
the femoral nerve. The pain disappears with flexion of the
thigh (Astley Cooper sign).
Many surgical techniques for the repair of femoral hernias
have been described; but because these hernias are rarely
reported in children, no large series exist to objectively compare them. The McVay hernia repair (approximation of
Cooper’s ligament to the conjoined tendon) is probably the
most widely used technique. Laparoscopy has recently been
described for the diagnosis and repair of femoral hernias.
The most common error made regarding femoral hernia
repairs is misdiagnosis. This results in inadequate treatment
at the first operation and patients being subjected to repeated
procedures for a “recurrent” hernia. In patients with negative
explorations for suspected indirect inguinal hernias, the diagnosis of a femoral hernia should be considered. The most
significant operative complications are recurrence and intestinal compromise secondary to strangulation.
Spigelian Hernias
Fewer than fifty cases of Spigelian hernia in the pediatric age
group have been reported in the literature. These hernias
occur lateral to the rectus abdominus muscle along the semilunar line. In adults and older children, these hernias are
thought to be the result of trauma, neoplasms, or prior
abdominal operations. However, the occurrence of Spigelian
hernias in neonates has raised the possibility of a congenital
etiology. In these patients, associated findings of inguinal
hernias and cryptorchid testes have been described.
The diagnosis of a Spigelian hernia can be made clinically
when a bulge is present along the semilunar line. This is often
a subtle finding because of the overlying intact external
S.J. Timmapuri and R. Prasad
oblique muscle, which prevents complete herniation of
abdominal contents. If unclear clinically, US, CT or MRI
sometimes aids in confirming the diagnosis. Once diagnosed,
Spigelian hernias should be repaired in order to alleviate associated discomfort and prevent incarceration. Primary repair is
almost always possible in children and can be accomplished
through a transverse incision directly over the fascial defect.
Prosthetic material is sometimes necessary for larger defects.
Laparoscopy has been found to be very useful for both diagnosis and treatment. The hernias can be repaired entirely laparoscopically or by using laparoscopy as an adjunct to identify
the exact site of the hernia and guide incision placement.
Because of the often subtle nature of Spigelian hernias,
the diagnosis is challenging. In patients without an obvious
bulge on examination but with a good history, radiologic
studies might be helpful. Recurrence after repair is rare,
especially with small defects. Other complications relate to
intestinal ischemia secondary to incarceration.
Lumbar Hernias
Although approximately 10% of all lumbar hernias are congenital in origin, congenital lumbar hernias (CLH) account for
the majority of cases in children. In older children and adults,
acquired lumbar hernias result from trauma, infection or prior
surgery. The most common types are Grynfelt-Lesshaft hernias and Petit hernias. Grynfelt-Lesshaft hernias occur in the
superior lumbar triangle, bordered by the inferior aspect of the
12th rib, the internal oblique muscle, and the quadratus lumborum. Petit hernias occur in the inferior lumbar triangle,
whose borders are the external oblique muscle, the latissimus
dorsi muscle, and the iliac crest. Combinations of both hernia
types are occasionally seen and result in a large defect.
The etiology of CLH is not completely clear. Possible factors are developmental anomalies, such as aplasia of lumbar
muscles, nerve entrapment, and increased pressure due to an
intra-abdominal mass. Most present in the first year of life.
They tend to be unilateral and have well-defined borders. The
diagnosis is made on physical examination and the classification is based on the anatomic location. Associated anomalies
are present in nearly two-thirds of patients with CLH. These
include lumbocostovertebral syndrome, caudal regression
syndrome, diaphragmatic hernia, ureteropelvic junction
obstruction, cloacal exstrophy, and, rarely, lipomeningocele.
After the associated medical conditions are properly
addressed, repair of CLH is recommended to prevent incarceration. As with other hernia types, primary repair is the preferred method; however, especially with combined defects,
the hernia can involve the entire lateral abdominal wall, and
repair with prosthetic material is often necessary. Recurrence
is rare and usually occurs in patients with other significant
69 Uncommon Hernias
Traumatic Abdominal Wall Hernias
Traumatic abdominal wall hernias (TAWH) are rare defects
which result from high- or low-velocity blunt trauma. The
actual injury is caused by an object projecting enough force to
cause damage to muscle and fascial layers without penetrating the skin. The elastic nature of the skin allows it to remain
intact while the other abdominal wall layers are disrupted.
Three types of TAWH have been described: Type I hernias are
small defects caused by blunt trauma (handlebar injury), Type
II result from high-velocity traumas (motor vehicle accidents,
falls) and tend to be larger, and Type III, which typically result
from deceleration injuries and involve herniation of intraabdominal contents. TAWH are usually seen in the lower
abdomen but can occur anywhere on the abdominal wall.
Not all TAWH are evident immediately following the
traumatic event but rather can present in a delayed fashion
weeks or months later. To make the diagnosis of TAWH,
patients should have had clear abdominal wall trauma without evidence of a hernia prior to the injury. The diagnosis is
usually based on history and physical examination, however
swelling or hematoma in the area of injury can obscure the
bulge of the hernia, in which case radiographic studies (plain
abdominal films, US, CT) can be useful for confirming the
diagnosis and excluding other injuries.
Although all patients with abdominal trauma must be carefully evaluated, children with the smaller Type I defects rarely
have concurrent intra-abdominal injuries. Repair is indicated
whenever the diagnosis is made. Other traumatic injuries
must be appropriately addressed, sometimes necessitating
laparotomy. However, in the absence of other intra-abdominal
pathology, a local wound exploration with primary closure of
the hernia in multiple layers should be performed. Larger
defects might require the use of prosthetic material for an
adequate tension-free closure. Diagnostic laparoscopy is used
in some patients to evaluate abdominal injuries and obviate
the need for a larger laparotomy incision. This approach
allows for a thorough abdominal exploration and subsequent
local hernia repair in patients with no additional injuries.
Rare hernias in the pediatric population are frequently
difficult to diagnose. Physical findings are vague and radiographic studies are unhelpful. Misdiagnosis of these defects
can have serious consequences such as bowel ischemia,
obstruction, and perforation. A clear understanding of the
anatomy, along with a preoperative management plan, aid in
the diagnosis and treatment of these uncommon hernias.
Summary Points
• Understanding the anatomy and repair techniques for various uncommon hernias is critical for appropriate diagnosis and management.
• Diagnosis of most hernias is based on history and physical examination.
• Misdiagnosis leads to complications, including bowel obstruction, incarceration, and perforation.
Editor’s Comment
One of the rarest hernias seen in children is the direct inguinal
hernia. Essentially all pediatric inguinal hernias are of the
indirect variety (patent processus) and therefore high ligation
is all that is necessary. It is very rare to discover that the floor
of the inguinal canal is deficient either before or during
an inguinal hernia repair. With the possible exception of a
second recurrence or a child with a known connective tissue
disorder, the use of an artificial material is not recommended
for inguinal hernia repair in a child because of the uncertain
long-term effects and the possibility of injury to adjacent
structures. Rather, a traditional technique, such as the McVay
Cooper ligament repair, is favored.
Spigelian hernias are frequently considered but rarely
found. Patients will sometimes provide a history that is textbook, only to have intact fascia by physical examination,
imaging studies, and even local exploration. It can be very
frustrating for all involved. Although MRI and laparoscopy
provide the best available techniques for confirmation of a hernia, no study or combination of studies is especially accurate.
The eponymous inguinal hernias are mostly of historical
interest as they rarely cause true clinical mayhem – when performing an inguinal hernia repair, the surgeon must be prepared to deal with any number of surprises. The one exception
is the Richter’s hernia, which can be easily missed when one
assumes that incarcerated hernia has been ruled out because
of the absence of obstructive symptoms. The consequences of
this error, bowel necrosis and perforation, can be dire. Imaging
studies can also be deceiving, reinforcing the adage that,
when in doubt, the patient should be explored.
Diastasis recti is not a true hernia and should never be operated upon, but parents often need to be repeatedly reassured.
Likewise, epigastric hernias (epiploceles) should only be
repaired if they become large or symptomatic. Despite being
true hernias, they are almost always tiny, allowing only a small
amount of properitoneal fat to herniated, and are thus ­generally
S.J. Timmapuri and R. Prasad
harmless. Finally, incisional hernias can occur whenever a
surgical procedure has been performed and are usually easily
confirmed on physical examination. The exception is the rare
trocar site hernia, which can be difficult to diagnose. The
combination of pain and a lump at a trocar site should usually
prompt surgical exploration to rule out a hernia.
Parental Preparation
−− Risk of infection, bleeding (hematoma), error in diagnosis, and recurrence.
−− Some abdominal wall hernias are rare and difficult to
confirm or exclude even with sophisticated imaging
Differential Diagnosis
• Littre’s hernia (contains Meckel’s diverticulum)
• Amyand’s hernia (contains appendix)
• Richter’s hernia (incarcerated antimesenteric portion of bowel)
• Femoral hernia
• Lymphadenopathy
• Abscess
• Spigelian hernia
• Traumatic abdominal wall hernia
• Lumbar hernia
• Traumatic abdominal wall hernia
Diagnostic Studies
Plain radiographs
Preoperative Preparation
□□ Informed consent
□□ Careful evaluation of preoperative studies
Technical Points
• Although not always possible, accurate preoperative
diagnosis allows appropriate operative planning.
• Careful evaluation of involved bowel is critical to
identify an ischemic or perforated segment.
• Laparoscopy is a useful adjunct in the diagnosis and
management of unusual hernias.
Suggested Reading
Chan KW, Lee KH, Mou JWC, et al. The use of laparoscopy in the
management of Littre’s hernia in children. Pediatr Surg Int.
DeCaluwe D, Chertin B, Puri P. Childhood femoral hernia: a commonly
misdiagnosed condition. Pediatr Surg Int. 2003;19:608–9.
Fakhry SM, Azizkhan RG. Observations and current operative management of congenital lumbar hernias during infancy. Surg Gynecol
Obstet. 1991;172:475–9.
Goliath J, Mittal V, McDonough J. Traumatic handlebar hernia: a rare
abdominal wall hernia. J Pediatr Surg. 2004;39:e20–2.
Livaditi E, Mavridis G, Christopoulos G. Amyand’s hernia in premature neonates: report of two cases. Hernia. 2007;11:547–9.
Losanoff JE, Richman BW, Jones JW. Spigelian hernia in a child: case
report and review of the literature. Hernia. 2002;6:191–3.
Messina M, Ferrucci E, Meucci D, et al. Littre’s hernia in newborn
infants: report of two cases. Pediatr Surg Int. 2005;21:485–7.
Temiz A, Akcora B, Temiz M, et al. A rare and frequently unrecognized
pathology in children: femoral hernia. Hernia. 2008;12:553–6.
Tycast JF, Kumpf AL, Schwartz TL, et al. Amyand’s hernia: a case
report describing laparoscopic repair in a pediatric patient. J Pediatr
Surg. 2008;43:2112–4.
Wakhlu A, Wakhlu AK. Congenital lumbar hernia. Pediatr Surg Int.