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Fig. 10 Post-traumatic
diaphragmatic hernia, same
clinical case as Fig.6,
intraoperative view.
Diaphragmatic defect
Fig. 11 Post-traumatic
diaphragmatic hernia, same
clinical case as Fig.6,
intraoperative view. Defect
closed with suture
G. D. Tebala et al.
The initial step of the operation is to conduct a thorough examination of the
abdominal cavity and an exploration of the site of the hernia. Once the diagnosis
and site have been conrmed, the herniated viscera are slowly and carefully reduced
into the abdomen. All adhesions within the sac and to the diaphragm must be
divided. The closure of the diaphragmatic defect is usually carried out with single
stitches or a running suture with a non-absorbable or slowly absorbable material
(such as polydioxanone). Before closing the diaphragm, the defect must be inspected
to ensure that the pleura is not damaged. In case of a pneumothorax, it is advisable
to ask the anaesthetist to increase the tidal volume and expand the lung before nishing the suture. A chest tube should be placed either at this stage or later on in the
operation but must be in place before extubating the patient.
Large hernias may require the use of a mesh to bridge the defect or to reinforce the suture if the edges can be approximated. Obviously, the mesh must be
centred on the defect. Some authors suggest suturing the mesh to the previous
diaphragmatic suture and gluing the edges of the mesh to the diaphragm [35]. An
alternative technique is to suture the four corners of the mesh to the diaphragm
and glue the rest, but it is important to be exible and adapt your operative technique based on the location and features of the diaphragmatic hernia. The use of

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tacks to x the mesh to the diaphragm is not advisable, particularly medially, due
to the risk of cardiac injury. A systematic review identied 23 cases of cardiac
injury due to diaphragmatic tacks, with a mortality of 48% [36]. Reducing the
abdominal pression during the suture and positioning of the mesh may be of
great help to decrease the tension on the surface of the diaphragm and allow an
easy closure of the defect.
The use of mesh to reinforce diaphragmatic defects has always been a controversial question, and there remains wide disagreement. A recent RCT published in
2020 on the use of suture vs. absorbable vs. non-absorbable mesh did not show any
advantage with the use of mesh to repair large hiatus hernias [37], but the sample
size was not very large. A meta-analysis published in the same year on more than
300 pooled patients yielded similar results [38]. It is not clear if these results can be
extended to the repair of diaphragmatic non-hiatus hernias.
The treatment of trans-hiatal post-oesophagectomy hernias can be performed
laparoscopically with great efcacy and low risk [39]. Some studies suggest placing a chest tube prior to induction of pneumoperitoneum [39], but in our experience, a chest drain can be inserted at any time during or after the procedure,
particularly if the anaesthetist reports high respiratory pressure and low compliance. The rst step of the operation is an adhesiolysis, to mobilise the contents of
the hernia, while being very careful not to injure the gastroepiploic vessels as they
represent the only source of vascular supply to the gastric tubule. For the same
reasons, the dissection of the herniated bowel must be performed close to the
crura [40]. After reducing the hernia, the repair of the hiatus is achieved through
an anterior and posterior cruroplasty with non-absorbable or slowly absorbable
sutures, possibly with the use of non-absorbable pledgets to avoid muscular tears
[9]. Some studies advocate the use of a biological mesh to plug the enlarged hiatus
[41] or to reinforce a weak diaphragmatic suture or residual defect [39]. Most of
these repairs can be done laparoscopically or robotically, but with a low threshold
for conversion [39].
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3 Congenital Hernias
3.1 Introduction
Abdominal wall defects represent a broad spectrum of congenital anomalies, varying from benign umbilical cord hernias to lethal conditions.
The two most common anomalies included in this group are omphalocele and
gastroschisis [42–44]. Both are usually diagnosed during pregnancy via foetal ultrasound, and their treatment requires assistance in a high-volume tertiary centre with
immediate access to high-risk obstetric services, neonatology and paediatric surgery [43, 44].
Although both conditions affect the umbilical area, the underlying pathology,
outcomes and associated abnormalities are different, and therefore treatment of the
two disorders is distinct (Table2).

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Table 2 Congenital abdominal wall defects’ characteristics
Characteristic
Location of the defect Umbilicus Right of umbilicus
Sac Present Absent
Extraintestinal-associated anomalies Common (40–80%) Uncommon (5–15%)
Bowel atresia Uncommon 6–28%
Bowel motility Typically preserved Impaired
Omphalocele
Gastroschisis
G. D. Tebala et al.
3.2 Omphalocele
3.2.1 Anatomical Definition andEpidemiology
Omphalocele (exomphalos) is a herniation of viscera through a midline abdominal
wall defect [45]. This anomaly occurs at the umbilical ring, and typically, the herniated viscera are covered by a three-layer sac composted by an inner peritoneal
layer, Wharton’s jelly and an external amniotic layer [43, 46]. The presence of the
sac is the main feature differentiating omphalocele from gastroschisis. With
omphalocele, the herniated organs are protected from the irritant effect of the
amniotic uid, and intestinal motility is therefore typically preserved, while in
gastroschisis, bowel exposure to the amniotic uid results in gastrointestinal dysmotility and functional impairment [46]. Omphalocele most commonly contains
the small bowel, but it can include other abdominal organs, such as liver, colon,
stomach, bladder, spleen and gonads, and its size can range from 2 to 10cm. The
term giant omphalocele is used for defects larger than 5cm. Association with other
anomalies is frequent, varying from 40 to 80%. The anomalies can include cardiac
(7–47%), respiratory (17–60%), musculoskeletal (4–25%), genitourinary (6–20%),
gastrointestinal (3–20%) and central nervous system (4–30%). Sometimes omphalocele may be present as part of a genetic disorder or syndrome (3–20%) such as
Beckwith-Wiedemann syndrome (macroglossia, gigantism, hypoglycaemia,
omphalocele, increased risk of childhood cancer), pentalogy of Cantrell (defects to
the midline abdominal wall, lower sternum, anterior diaphragm, diaphragmatic
pericardium and some form of intracardiac defect), OEIS complex (omphalocele,
exstrophy of the bladder, imperforate anus and spinal anomaly) and trisomy 12, 18
and 21 [42, 44–53]. Morbidity in children with omphalocele is mostly due to associated congenital anomalies, and the long-term outcomes are directly related to
these rather than to the abdominal defect itself, although the size of the defect has
recently been demonstrated to be an independent predictor of neonatal morbidity
and mortality [47–49]. The incidence rate of omphalocele is approximately 1–2 per
10.000 live births, although this number is higher if elective abortions and foetal
deaths are taken into account (1in 1.000–4.000) [52]. An association has been
observed with advanced or young maternal age, black infants, maternal obesity and
maternal glycaemic control disorders. The estimated survival rate for isolated
omphalocele is 50–90%; however, it signicantly decreases when concurrent
anomalies are present [45].

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3.2.2 Diagnosis
The diagnosis is usually made prenatally, most commonly in the late rst or second
trimester. Since the midgut undergoes physiologic herniation during the sixth week
of gestation and normally does not fully return into the abdomen before the 11th or
12th week, denitive diagnosis of omphalocele should not be made before the 12th
week of gestation [42, 46, 55]. Elevated maternal serum α-fetoprotein should raise
suspicion, and prenatal ultrasonography (US) is the gold standard imaging modality
for detecting ventral abdominal wall defects [55]. When an omphalocele is diagnosed, prenatal evaluation should focus on detecting potential associated anomalies,
as these are the major determinants of outcome. Foetal magnetic resonance imaging
(MRI) is a valid method to assess these anomalies and allow prediction of postnatal
morbidity, and it can particularly be useful in cases where the diagnosis is not evident on prenatal US [55]. Karyotype analysis is also indicated when an omphalocele
is suspected to rule out numerical chromosomal aberrations [45].
3.2.3 Management
After diagnosis of omphalocele, delivery should be carried out at a tertiary centre
where neonatological and pediatric surgical support is available. Full-term delivery
is recommended unless otherwise indicated for obstetric reasons or foetal distress
[43, 44]. No studies have demonstrated a clear benet of one route of delivery over
another, and this continues to be a topic of debate. Vaginal delivery has been demonstrated to be safe and feasible in children with small defects, while some advocate
for caesarean section in cases of giant omphalocele, due to the risk of sac rupture
during vaginal delivery, or when the liver is herniated, to avoid hepatic trauma [52].
Further prospective studies are needed to cast light on this issue in order to make
valid recommendations. As associated cardiopulmonary anomalies are the principal
source of morbidity, the initial management of newborns with omphalocele should
be focused on evaluating cardiorespiratory function, and support provided if necessary. Fluid loss is common in these children (although less so compared to gastroschisis), and uid balance status must be assessed, and uid resuscitation provided
where necessary. An oro- or nasogastric tube should be placed for gastric decompression, and the sac should be kept moist and protected with saline-soaked gauze
[56]. Antibiotics are typically administered for the rst 48h after birth in newborns
with surgical issues to rule out sepsis and should be discontinued if cultures are
negative after that period. Antibiotic treatment may not be necessary in infants with
intact omphalocele, and course duration should be as short as possible in all cases
[46]. Denitive surgical correction is not an emergency in children with omphalocele with an intact sac, and so the rst step after initial stabilisation is thorough
evaluation in order to detect and assess potential associated anomalies. This includes
almost invariably renal ultrasound, echocardiography, blood glucose level and
karyotype analysis [57, 58]. Surgical evaluation is mandatory to establish the appropriate management in order to reduce the herniated viscera and repairing the
abdominal defect. Intra-abdominal pressure levels should guide the surgical repair
strategy in order to avoid abdominal compartment syndrome, which is the most

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G. D. Tebala et al.
threatening complication in omphalocele correction, causing impaired venous
return, decreased pulmonary compliance, renal failure and bowel ischaemia [59].
Surgical management is dictated by the size of the defect, the degree of visceraabdominal disproportion, the presence or absence of an intact sac and the cardiopulmonary status of the infant. In children with small defects, primary reduction and
closure may be attempted. This is performed by excising the sac at the skin and
fascia edge, with careful identication and ligation of the umbilical vessels. Then,
after reduction of the herniated viscera, and separation of the skin from the deep
fascia layers, the fascial edges are closed transversely with running or interrupted
absorbable sutures, and the overlying skin can be closed with a purse-string suture,
reconstructing the umbilicus [56, 58]. Although some surgeons advocate leaving the
sac intact and repairing the fascia and skin over it, in most cases, sac excision is
preferred to allow complete abdominal exploration. If primary closure is not achievable while also maintaining appropriately low intra-abdominal pressure levels, a
staged repair can be undertaken. In staged repair, the gradual reduction of the herniated viscera allows the abdominal wall to stretch in order to accommodate the content of the sac avoiding excessive intra-abdominal pressures. As gastrointestinal
function and motility are not impaired in children with omphalocele, unlike those
with gastroschisis, enteral feeding can be administered while awaiting denitive
closure of the ventral abdominal wall. The Schuster technique is the main staged
repair strategy currently in use. It is made using a prosthetic ‘silo’ to gradually
reduce the herniated viscera into the abdomen. After excising the sac, the silo is then
sewn to the fascia or to the muscular and fascial layers of the whole abdominal wall
using a running non-absorbable suture. Alternatively, the silo can be sewn over the
sac, or the sac itself can be used as a silo if it is free from the underlying viscera
(amnion inversion technique). The silo is sequentially and progressively tightened
over the course of days and weeks, typically at the bedside, without anaesthesia, to
gradually reduce its content into the abdomen with the goal of fascial closure [43,
46, 56, 57, 60, 61]. The silo can be hung over the patient’s bed to allow gravity to
enhance visceral reduction.
In some cases, signicant cardiopulmonary alterations or giant defects may lead
the caregivers to prefer initial non-operative management. Topical escharotic therapy, also known as the ‘paint and wait’ technique is a non-surgical strategy, described
for giant omphaloceles, that can postpone surgical repair for months, providing time
to let the child’s body and lungs grow. In this case, a thin layer of escharotic agent
(a corrosive paste that promotes eschar formation) [58] is applied over the sac and
wrapped with sterile gauze, and this procedure is repeated daily until the sac is
replaced by granulation tissue (usually 3–4weeks), which will then be gradually
covered by intact skin leaving a ventral hernia that will likely need repair at a later
stage [52]. Different topical agents have been described and might be considered.
These include silver sulfadiazine and other silver-based solutions, povidone-iodine
solution, neomycin and polymyxin/bacitracin ointments [61–64]. Each of these
agents promotes the formation of granulation tissue over the omphalocele membrane [61]. The escharotic agent can be applied also by the parents at home, allowing the infant to be discharged from the hospital when appropriate, until ready for

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delayed abdominal wall closure. Other techniques described for giant omphalocele
treatment include the use of a biologic or synthetic bridging mesh to cover the
defect (Gross technique) or a temporary vacuum dressing (negative pressure wound
therapy) [65, 66]. No single method is universally applicable to all cases, which has
led to the wide variety of techniques that are performed [58, 61].
Abdominal wall defects derive embryologically from an interruption of the physiological rotation of the intestines during foetal development, and infants with
omphalocele will therefore have abnormal bowel rotation and xation. As a result,
patients with abdominal wall defects are at risk of developing midgut volvulus. The
risk is higher in children with omphalocele, particularly in those born with an intact
sac. This may be due to fewer adhesions being formed after reduction, which would
normally prevent the intestines from rotating. Therefore, some surgeons recommend performing a prophylactic Ladd procedure during or after closure to decrease
the risk, although other studies do not support this practice [43, 54, 58, 67].
Infants with omphalocele may suffer from complications associated with the disease itself or with the method of repair. The most frequent complication is sepsis
[68]. Primary repair can lead to hernia, particularly if under tension. Separation of
the abdominal wall layers can lead to seroma or haematoma development in the
subcutaneous space, and damage to perforating vessels can lead to skin necrosis.
The use of a silo or a bridging mesh can damage fascial edges and lead to infection.
With regard to topical therapies, the chronic use of iodine can lead to hypothyroidism, and in general, escharotic agents can lead to ruptured omphalocele [62].
3.2.4 Ruptured Omphalocele
Omphaloceles can have a ruptured membrane. These ruptures can be dened as
primary (prenatal) or secondary (postnatal) [51]. Tears or rupture result in exposure
of the herniated viscera to the irritating effect of amniotic uid or environmental
stimuli as in gastroschisis, and postnatal management of the defect in these two
conditions is therefore similar. Both small and giant omphaloceles may have a ruptured membrane, although the risk of rupture is greater in giant defects. Differential
diagnosis between prenatal ruptured omphalocele and gastroschisis may be challenging, but it is of vital importance to determine the correct diagnosis in order to
identify potential associated anomalies. The method of delivery has not been associated with a change in rate of rupture, and therefore caesarean section is not routinely
recommended [68]. Postnatal rupture can occur during medical or surgical treatment of omphalocele, including during escharotic therapy. The rate of rupture has
been estimated at between 7 and 15% [51, 68].
In omphalocele, rupture represents an emergency and necessitates immediate
intervention. After initial cardiopulmonary stabilisation, management of the neonate with ruptured omphalocele should focus on maintaining euvolemia and normothermia. As in children with gastroschisis, these patients suffer from signicant
third-space and evaporative uid losses, meaning intravenous access should be
obtained, and uid resuscitation must be promptly started. The abdominal defect
should be covered with moist sterile gauze, or the lower half of the infant should be
placed in a sterile plastic bag during initial stabilisation, and an oro- or nasogastric

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G. D. Tebala et al.
tube should be placed for gastric decompression [56, 57]. Care should be taken in
avoiding injuries to the liver and spleen when the membrane is ruptured. Broadspectrum antibiotic prophylaxis should be started in infants with ruptured omphalocele [69].
Primary closure is appropriate when the abdominal wall defect is small to moderate in size and it obviates the morbidity of the multiple procedures required by staged
or delayed repair. When primary closure is not feasible, the use of a synthetic or
biological mesh bridge (Gross technique) or a prosthetic silo (Schuster technique)
allows the gradual reduction of the herniated viscera serving also as a barrier against
external agents. In large defects, vacuum-assisted repair has also been described as
an appropriate method to progressively gain abdominal domain. In case of ruptured
omphaloceles with minimal loss of membrane, topical escharotic therapy has been
described after re-approximation of the membrane with absorbable sutures [51].
3.3 Gastroschisis
3.3.1 Anatomical Definition andEpidemiology
Gastroschisis is a herniation of the bowel, and in some cases, other abdominal
organs through a ventral abdominal wall defect normally located 1–2cm right to the
umbilicus, and it does not have a membranous covering [56] (Fig.12). Typically,
gastroschisis is an isolated nding and lacks congenital associated anomalies. The
absence of a sac exposes the herniated viscera to the irritant effect of amniotic uid
during gestation, resulting in gastrointestinal dysmotility, which is the main cause
of morbidity in gastroschisis.
Fig. 12 Infant with simple
gastroschisis. The
abdominal wall defect is
located to the right side of
the umbilical cord insertion

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Intestinal atresia is the most common associated anomaly in infants with gastroschisis (6–28% of the children), and it is probably secondary to trauma of the bowel
against the abdominal wall or vascular compromise due to segmental volvulus
[42, 46].
The incidence of gastroschisis is approximately 1–5 per 10.000 live births [43].
The most signicant risk factor seems to be young maternal age; others include
tobacco, alcohol, recreational drug and some decongestant use [70–72]. These
agents (smoke, cocaine, amphetamines, decongestants) are thought to have sympathomimetic effects during embryogenesis, promoting the vascular accident
hypothesis. Other theories around embryogenesis of gastroschisis include failure
of lateral body wall folding, regression of the right umbilical vein with associated
localised paraumbilical tissue weakness and vascular accidents of the vitelline
artery [73].
In gastroschisis, the proximal intestine is typically dilated and suffers from dysmotility. In severe cases, a large portion of bowel is compromised, which can lead
to short bowel syndrome, partly due to the corrective surgery required [73]. In some
cases, the abdominal wall defect closes before birth, strangulating the bowel passing
through it. This phenomenon can lead to ischaemia, necrosis and, potentially, amputation of the bowel and is referred to as ‘vanishing gastroschisis’ [74]. Gastroschisis
is classied into simple and complex based on the presence of associated bowel
conditions (atresia, matting, necrosis, perforation, ischaemia, volvulus, vanishing
gastroschisis) [75]. Complex gastroschisis (17% of cases) has been associated with
higher rates of morbidity and mortality and with longer duration of total parenteral
nutrition and time to full enteral feeding [76].
3.3.2 Diagnosis
Unlike omphalocele, infants with gastroschisis tend not to be born at term but rather
at an average gestational age of 35–36weeks, and intrauterine foetal death rate is
approximately 5% [77, 78]. Gastroschisis is associated with higher maternal
α-fetoprotein levels than omphalocele (seven times normal vs. four times normal)
[79]. Prenatal US is a sensitive test that can detect the free-oating bowel with no
covering membrane outside of the foetal abdomen; the umbilical cord insertion site
appears paraumbilical [55].
Although gastroschisis is typically an isolated nding, approximately 5–15%
of the newborns affected will have associated extraintestinal congenital anomalies; therefore, it is important to run tests to rule out additional malformations
[55, 73]. Serial prenatal US is vital to monitor bowel development and viability,
as intestinal loops may appear progressively thickened as a result from irritation
caused by exposure to amniotic uid. Intra-abdominal bowel dilation and polyhydramnios have been associated with underlying intestinal atresia and gastric
dilation with neonatal death [80]. Increasing dilation and echogenicity of the
bowel may be a sign of impending ischaemia of the bowel. A contrast enema
should be performed, when intestinal atresia is suspected or conrmed, to determine the level of the lesion [46].

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3.3.3 Management
Vaginal delivery is preferred in gastroschisis, as in omphaloceles, unless caesarean
section is indicated for foetal distress or obstetric reasons. The current literature
shows no differences in outcomes between vaginal and caesarean delivery in children with gastroschisis [73]. A recent meta-analysis demonstrated no difference in
terms of overall mortality, feasibility of primary repair, incidence of necrotising
enterocolitis, sepsis, time to full feeding or duration of hospital stay [81]. Currently,
there is debate over the appropriate time of delivery in gastroschisis. Although the
policy in most centres consists of waiting until the natural onset of labour, some
groups recommend preterm delivery as it is associated with shorter duration of
exposure to amniotic uid and, therefore, lower degree of intestinal dysmotility,
shorter time to rst enteral feed and decreased risk of neonatal sepsis [82, 83].
Immediately after birth, the herniated viscera must be protected from the external environment, so humidity and temperature can be kept constant. The most common option for achieving this is to use a Lahey bag (or ‘bowel bag’). This is a sterile
bag into which the lower half of the newborn is placed; the bag is then loosely tied
around the chest. The bowel must be positioned in a way that protects the mesentery, and the vessels therein contained from twisting or kinking against the abdominal wall to preserve the intestinal blood ow. Intravenous access should be obtained
in order to stabilise the newborn with uids when necessary and in anticipation of
starting total parenteral nutrition soon after birth. Fluid resuscitation must be judicious, to prevent pulmonary oedema and the subsequent need for mechanical ventilation, and should be guided by vital parameters, capillary rell time, urine output
and acid-base status [73]. Primary surgical closure of the defect is the strategy of
choice if achievable without causing abdominal compartment syndrome. Normally,
it is obtained suturing the fascia after reduction of the viscera in the abdomen. More
recently a ‘sutureless’ method has gained popularity. This approach consists of coiling or hanging the umbilical cord remnant over the abdominal wall defect and placing a tight adhesive dressing over it, and the dressing is then changed every few days
until the fascial defect has closed (Fig.13). This method can be performed at the
bedside, and it does not require general anaesthesia. Some infants may initially
develop an umbilical hernia, but most of these will close over time [84]. If the herniated bowel mass is too large or too oedematous to perform primary reduction and
closure, a prosthetic silo can be placed over the viscera. The infant should then be
transferred into a NICU, where the silo can be tightened once or twice daily at the
bedside, with a small amount of sedation, to progressively reduce its contents into
the abdomen. During reductions, constant attention must be given to the haemodynamics of the newborn [56, 73]. Once completely reduced, the infant can then
undergo primary surgical closure or ‘sutureless’ repair. Regardless of the method
chosen, the herniated bowel should be inspected before reduction to check for the
presence of atretic segments before attempting reduction. In children with gastroschisis complicated by atresia, perforation or necrosis, a bowel resection or creation
of stoma may be necessary. At birth, the bowel is usually too oedematous to safely
undergo resection and immediate anastomosis to repair intestinal atresia. Therefore,
these are treated after 4–6weeks, when anastomoses are thought to be more secure

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a
b
c
Fig. 13 Primary reduction of gastroschisis. The bowel loops are carefully and progressively
reduced into the abdomen (a, b), the umbilical cord remnant is then hung above the defect, and a
tight adhesive dressing is placed over it (c)
[43, 46]. The bowel is typically malrotated in infants with gastroschisis. However,
the presence of intestinal adhesions is signicant enough that volvulus is a rare
event [85]. Mortality from gastroschisis and its associated complications has
decreased to less than 10% in most series due to recent advances in prenatal diagnostic accuracy, neonatal critical care and surgical management [86]. Bowel dysmotility and ileus affect these children long after surgical correction, resulting in
hospitalisation for weeks to months. Total parenteral nutrition must be started soon
after birth, anticipating intestinal failure and preventing malnutrition. Enteral feeding is then slowly and progressively initiated, and the infant is monitored to evaluate
tolerance. The Gastroschisis Prognostic Score (GPS) is a validated scale performed
at the bedside shortly after birth that evaluates the presence and severity of bowel
necrosis, matting, atresia and perforation in order to predict the duration of hospitalisation and total parenteral nutrition [87] (Table 3). To optimise care for these
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