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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
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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 conrmed, 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 identied 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 efcacy 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 [4244]. 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 (Table2).
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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
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3.2 Omphalocele
3.2.1 Anatomical Definition andEpidemiology
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 10cm. The term giant omphalocele is used for defects larger than 5cm. 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, 4453]. 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 [4749]. 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 (1in 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 signicantly 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, denitive 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 benet 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 48h 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]. Denitive 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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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 identication 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 denitive 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, signicant 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 3–4weeks), 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 [6164]. 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 dened 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 signicant 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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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 andEpidemiology
Gastroschisis is a herniation of the bowel, and in some cases, other abdominal organs through a ventral abdominal wall defect normally located 1–2cm 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 signicant risk factor seems to be young maternal age; others include tobacco, alcohol, recreational drug and some decongestant use [7072]. 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 classied 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 35–36weeks, 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 conrmed, 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 rell 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 4–6weeks, 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 signicant 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