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Gastroschisis With Liver Herniation: Facts And Challenges

Elena Ţarcă¹,², Bogdan Savu¹,², Sandu Gabriel Aprodu¹,², Dan George Goţia¹, Sebastian Ionescu³

¹„Gr. T Popa” University of Medicine and Pharmacy, Discipline of Pediatric Surgery and Orthopedics, Iasi, Romania

²„Sf. Maria” Emergency Children Hospital, Department of Peditric Surgery and Orthopedics, Iasi, Romania

³„Carol Davila” University of Medicine and Pharmacy, Discipline of Pediatric Surgery and Orthopedics, Bucharest, Romania


Abstract

Introduction: Gastroschisis is a congenital malformation in which the abdominal contents freely protrude through a defect involving all the anatomic layers of the anterior abdominal wall, most often to the right of the umbilical cord, which is normally inserted and formed. The small intestine, a large part of the colon, sometimes parts of the stomach, duodenum, fallopian tube and uterine horn, ovary or undescended testis, or the bladder apex usually stick out. Liver herniation has been extremely rare, and therefore some authors argue that these cases are actually ruptured omphalocele cases.

Material and methods: We conducted a retrospective study of 114 cases of gastroschisis treated in the Pediatric Surgery Department of “Sfânta Maria” Emergency Children’s Hospital of Iaşi over a period of 23 years, namely between 1990 and 2012.

Results: According to Molik’s classification, 24 of these cases were complex, whereas the other 90 patients only suffered from simple gastroschisis. Four of them had gastroschisis with liver herniation (3.5%), three of whom only had the left liver lobe protruding and the fourth had the entire liver sticking out. Although the degree of visceral-abdominal disproportion was considerable and the cases were obviously complex (all the patients died), since no intestinal atresia or perforations were associated, according to Molik’s classification, they were included in the simple gastroschisis group, thus increasing the mortality rate in this group. The mortality rate in the complex gastroschisis group was 66.7%, and the patients’ death occurred 15.33 days later, on the average, as compared to a mortality rate of 71.1% in the simple gastroschisis group, with the patients’ death occurring after 12.75 days, on the average.

Conclusions: We suggest that Molik’s classification be changed to also include gastroschisis with liver herniation among the complex gastroschisis cases, due to the undeniable visceral-abdominal disproportion, to the difficult integration of the protruding organs and to the patients’ guarded prognosis.

Key words: complex gastroschisis, liver, classification, mortality.


Correspondence

Elena Ţarcă
“Gr. T. Popa” University of Medicine and Pharmacy
“Sf. Maria” Emergency Children Hospital, Clinic of Pediatric Surgery and Orthopedics
Vasile Lupu Street, no 62., Iasi, Romania
Phone: +40765 485446,
e-mail: This email address is being protected from spambots. You need JavaScript enabled to view it.


Introduction

Gastroschisis is the result of the intrauterine intestine loop herniation through a small defect in the abdominal wall, usually located to the right of the normally implanted umbilical cord. The whole intestinal tract is usually gutted and floats freely in the amniotic fluid, with no sac to hold it. The ovaries, testicles or liver are less involved. The etiopathogenesis of this abdominal wall defect has been a subject of debate, yet most authors agree on being the outcome of a stroke involving the right omphalomesenteric artery [1], although other authors argue that a small number of cases may be due to an intrauterine ruptured omphalocele, which would also account for the occasional occurrence of liver herniation [2].

A thick inflammatory membrane covers the intestines, which are shortened and have a thickened mesentery. These factors are correlated to motility and nutrient absorption diminution. These changes are the result of a combination of factors, including prolonged contact with the amniotic fluid and intestine constriction due to abdominal wall defect. It was Moore who, in 1953, first distinguished between omphalocele and gastroschisis and formulated a theory on gastroschisis etiopathogenesis [3]: the normal embryological development of the anterior abdominal wall is possible by the ventral extension of the thoracic wall and of the lumbar myotomes. Therefore, any interruption or alteration of this extension may cause the occurrence of the gastroschisis-specific parietal defect.

In 1963 Duhamel suggests that gastroschisis would be some sort of minor celosomia, an anomalous embryonic mesenchyme differentiation from the lateral folds of the abdominal wall [4]. Thus, the anterior abdominal wall becomes more fragile, it breaks while the fetus is still in the uterus and it thus leads to intestinal evisceration. This process may be induced by a teratogenic agent with early direct or indirect action during organ genesis. In 1975 Shaw proves that in gastroschisis abdominal wall defect appears either between the 5th and the 10th weeks of intrauterine life, or close to birth. He continues Duhamel’s theory, yet he thinks that the mechanism is different: gastroschisis is a mere cord hernia and tear, after gastrulation process completion and abdominal wall forming, yet before complete umbilical ring closing [5]. He grounds his theory on a series of observations described in literature and concludes that gastroschisis is actually a ruptured omphalocele.

In 1981, Hoyme lays down a vascular theory according to which intrauterine thrombosis of the omphalo-mesenteric artery causes the parietal defect and associated intestinal atresia [1]. Intrauterine thrombosis of the omphalo-mesenteric artery leads to infarction and necrosis of the right side of the umbilical cord basis, and to intestine loop herniation in the amniotic cavity. The discontinuation of the proximal omphalomesenteric artery may also lead to the occurrence of intestinal atresia and stenosis, as well as other association anomalies: intestinal malrotation, gall bladder atresia, renal agenesis. This theory conflicts with the assumption according to which intestinal atresia in gastroschisis are caused by compressed intestine loops in the abdominal wall defect, which does not however account for the occurrence of other associated anomalies.

Hoyme agrees with Shaw’s theory, which claims that the borders of the cutaneous defect grow in the intrauterine healing process. This would explain the occurrence of an integumentary bridge between the umbilical cord and the gastroschisis orifice. Vascular theories are also supported by the fact that gastroschisis is more common in mothers who use vasopressor agents, such as nicotine, cocaine or pseudoephedrine, during their pregnancy [6]. Further progress achieved by experimental studies and antenatal imagining monitoring of the pregnancy will further clarify omphalocele and gastroschisis etiopathogenesis.

Thus, gastroschisis is a congenital malformation in which the abdominal contents freely protrude through a defect involving all the anatomic layers of the anterior abdominal wall, most often to the right of the umbilical cord, which is normally inserted and formed. The umbilical cord is intact, its implantation basis is narrow and it is usually separated from the parietal defect by a thin normal-looking tegument. The orifice is rather small, its diameter ranging between 1.5 and 5 cm, and its borders are smooth, regular and slightly extensible. Any amniotic membrane remains on the borders of the defect may indicate an intrauterine ruptured omphalocele. Both aponeuroses and muscles are normally developed.

The straight muscles of the abdomen are inserted at the xiphoid level and they merge at paraumbilical orifice level into a single skin-covered fibrous ring. The protruding organs are never covered by the sac, but they may adhere to one another and be covered by fibrin membranes. The small intestine, a large part of the colon (as it is not properly adjoined to the posterior abdominal wall), sometimes parts of the stomach, duodenum, fallopian tube and uterine horn, ovary or retained testis, or the bladder apex usually stick out.

Liver herniation has been extremely rare, and therefore some authors argue that these cases are actually ruptured omphalocele cases [7]. The more viscera sticking out from the peritoneal cavity the smaller the peritoneal cavity, which is due to insufficient intrauterine development in the absence of mechanical distension. The protruding intestine loops have a particular appearance, as they are thickened, stiff, dilated and shortened, peristaltic-free and covered by a yellow-greenish adherent inflammatory membrane called “peel” [8], which agglutinates the loops together and turns them into a tumor mass, which indicates plastic or chemical antenatal peritonitis. The intestine is always malrotated and it is not properly adjoined to the posterior abdominal wall. Congenital malformations associated to gastroschisis are uncommon. Nevertheless, intestinal atresia and digestive perforations have higher incidence rates (5%) as compared to omphalocele. Full intestinal necrosis or ischemia is sometimes detected.

Material and Method

We conducted a retrospective study of 114 cases of gastroschisis treated in the Pediatric Surgery Department of “Sfânta Maria” Emergency Children’s Hospital of Iaşi over a period of 23 years, namely between 1990 and 2012. Please note that our hospital is the only tertiary center in the Moldova area, where most neonates with congenital malformations are referred for treatment.

This study does not include the babies who, at their parents’ request or for medical reasons, were referred to other specialized hospitals in the country, or who died within a few hours from their delivery. This may have biased the assessment of the actual incidence of abdominal wall defects in the Moldova area. We took all the patients’ medical records out of the hospital’s archives, both those related to their first hospitalization and those related to their subsequent hospitalizations. For the 1990 through 2002 period, the patients’ records were tracked down according to the examination and hospitalization registers, whereas those for the 2002 to date period were found in the hospital’s computerized database by entering the illness code. Eight patients whose medical records were damaged or incomplete were excluded from the research.

Results

According to Molik’s classification, 24 of the 114 cases of gastroschisis were complex: ileale (13 cases) and/or colonic (7 cases) atresia and stenosis, persistent omphaloenteric duct (1 case), ileal loop necrosis (3 cases), ileocecal volvulus (3 cases) (Figure 1 and 2), whereas the other 90 patients only suffered from simple gastroschisis (Figure 3).

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Figure 1: Gastroschisis with protruding loop necrosis and underlying ileal atresia

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Figure 2: Complex gastroschisis (ileal atresia)

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Figure 3: Simple gastroschisis

The size of the anterior abdominal wall defect in gastroschisis neonates was 3.39±1.18 cm on the average. The intestine loops alone were protruding in 47.3% of the cases. Gastroschisis is an anterior abdominal wall defect through which the intestine loops, stomach, sometimes spleen and possibly urinary bladder or fallopian tubes and ovaries usually stick out. Yet, in the group of patients under survey in this research, we also came across four cases of gastroschisis with liver herniation (3.5%), three of whom only had the left liver lobe protruding and the fourth had the entire liver sticking out. These cases were included in the simple gastroschisis group (Figure 4).

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Figure 4: Gastroschisis with protruding liver

The surgical treatment, which consisted of the thorough washing of the protruding organs using lukewarm saline solution and antiseptic solutions, was performed in the operating room, with the patient under general anesthesia by oral-tracheal intubation. This was followed by the careful examination of the intestine loops in order to detect any associated stenosis or atresia. If the intestine loops were supple and showed no atresia, stenosis, perforations or necrosis, the gastroschisis was considered simple; otherwise, the gastroschisis was considered complex. 20 of the 24 patients with complex gastroschisis (83.3%) underwent concomitant surgical procedures such as ileostomas (Figure 5), colostomas, intestine resections and anastomosis.

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Figure 5: Ileostoma (complex gastroschisis)

Additional surgical procedures were applied to 9 (37.5%) of these patients due to their post-operative complications. A quarter of the complex gastroschisis patients suffered from a short intestine syndrome after the surgical procedures, and only one of these 6 patients survived. Only 10 (11.1%) of the simple gastroschisis patients needed further surgery for post-operative complications, such as occlusions due to agglutination and ileal bundles or perforations and eviscerations due to strained abdominal wall closing. Abdominal compartment syndrome as one of the main causes of post-operative death occurred in 11.1% of the simple gastroschisis patients (as compared to zero complex gastroschisis patients). The treatment of all these patients consisted of primary integration and per primam abdominal wall closing (Figure 6).

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Figure 6: Full viscera integration

The mortality rate in the complex gastroschisis group was 66.7%, and the patients’ death occurred 15.33 days later, on the average, as compared to a mortality rate of 71.1% in the simple gastroschisis group, with the patients’ death occurring after 12.75 days, on the average. If one analyzes the length of stay of the surviving patients depending on the complexity of their gastroschisis, one notices a significant difference, namely 42.87 days for the first hospitalization episode of complex gastroschisis patients versus 27.07 days for simple gastroschisis patients. However, this difference is not statistically significant (p=0.201).

Discussions

In 1963, Moore suggests a gastroschisis classification depending on the size of the parietal defect and on the appearance of the intestine loops [8]:

• antenatal type: the evisceration occurs early during the gestation period, the intestinal lesions are considerable (stiff and thickened loops, and chemical peritonitis lesions) and the peritoneal cavity is insufficiently developed

• perinatal type: the evisceration occurs later during pregnancy, the intestine loops are supple, the peritonitis lesions are minimal and the peritoneal cavity is almost normally developed

• intermediate type: combination of the two types above.

In 1977, the same author classifies gastroschisis into three categories depending on the size of the parietal defect [9]. These classifications are faithful to the clinical appearance, but they disregard the pathophysiological support of the condition. Moreover, the progress of ultrasound scanning allowed antenatal diagnosis setting as early as the first quarter of pregnancy, which refutes the existence of perinatal forms. In 1978, Lefort distinguishes among the different types of gastroschisis based on anatomic appearance and prognosis, and achieves a more practical classification [10]:

• type 1: normal color and length small intestine and colon, without or with minimum plastic peritonitis lesions, without atresia; excellent prognosis;

• type 2: normal length small intestine and colon, with chemical peritonitis lesions, with poor vascularization, without atresia; good prognosis;

• type 3: small intestine and colon with chemical peritonitis lesions, poor vascularization, small intestine and/or colon atresia or necrosis/perforation lesions, diminished total intestine length; the patient may survive with proper treatment;

• type 4: digestive tract almost completely necrotic; incompatible with life.

Relying on the associated intestinal anomalies and prognosis, Molik proposes a simple yet practical classification in 2001 [11]:

• simple gastroschisis;

• complex gastroschisis.

The second category is defined by the occurrence of intestinal atresia or stenosis, perforations or ischemic areas, which increase morbidity and mortality. These cases will obviously require longer mechanical ventilation and, hence, more hospital stay days, they will suffer from prolonged paralytic ileus and malabsorption, and therefore have poorer prognosis.

Molik’s classification is also useful as it allows informing the parents of their baby’s evolution, possible complications, length of stay and sometimes mortality. In a retrospective study conducted on 4344 gastroschisis cases in 2007, Arnold also referred to it as being simple or complex depending on whether atresia, intestinal stenosis, volvulus, loop necrosis or perforations occurred or not.

According to his findings, 10.9% of the cases were complex [12]. However, none of these classifications includes protruding liver gastroschisis, with or without intestine loop damage. According to literature, morbidity and mortality rates are higher in complex gastroschisis. According to Molik’s classification, this is due to intra-abdominal infections and sepsis, digestive hemorrhage, abdominal compartment syndrome or respiratory distress [11].

An interesting result of our research was the relatively equal mortality rates of the simple and complex gastroschisis neonates, which were 71.1% and 66.7%, respectively. This may be accounted for by the absence of the abdominal compartment syndrome in complex gastroschisis patients, who suffered visceral mass diminution due to atresia, necrosis and necessary intestinal resections. Nine of the 10 simple gastroschisis patients in whom the abdominal compartment syndrome was detected died.

Although in our study 20 of the 24 complex gastroschisis patients suffered primary resections and anastomoses or stomas, the post-operative complication rate (37.5%) was similar to other literature data. This was not the case with the mortality rate. A quarter of the complex gastroschisis patients suffered from a short intestine syndrome after the surgical procedures, and only one of these 6 patients survived, since intestines are not transplanted in Romania. Although the great majority of specialized publications describe gastroschisis as an anterior abdominal wall defect through which the intestine loops, stomach, sometimes spleen and possibly gall bladder or fallopian tubes and ovaries protrude, and claim that liver herniation only occurs in omphalocele, there are authors who describe gastroschisis cases with protruding liver and thus acknowledge the special complexity of the cases and usually poor prognosis [13].

A 2011 study conducted on a group of 117 gastroschisis cases reports a 6% incidence of protruding liver cases and a 43% survival rate of these patients as compared to 97% in the other gastroschisis patients [14]. In our research, we came across four cases of gastroschisis with liver herniation (3.5%), three of whom only had the left liver lobe protruding and the fourth had the entire liver sticking out. Although the degree of visceral-abdominal disproportion was considerable and the cases were obviously complex (all the patients died), since no intestinal atresia or perforations were associated, according to Molik’s classification [11], they were included in the simple gastroschisis group, thus increasing the mortality rate in this group (we think that this may have biased the selection).

The long-term evolution of gastroschisis patients is also closely connected with the occurrence of intestinal atresia, which is one of the most important poor prognosis factors. The occurrence of multilayered intestinal atresia, with damaged proximal intestine loop requiring extended resection or ileocecal valve removal, predisposes the patient to subsequent short intestine syndrome and malabsorption. As a general rule, in literature many authors recommend avoiding primary anastomosis and resection as first-line treatment and postponing it for a few weeks after the primary closing.

Conclusions

In our opinion, the complex gastroschisis approach preferred in our clinic was too aggressive, as most specialized studies recommend leaving the atresia and stenosis areas as they are and only dealing with them at a later time, when the patient is medically stable, the intestine loops have regained their suppleness and the patient may tolerate the surgical procedure better.

This would increase the chances of primary anastomosis and prevent stoma-related complications. A much feared complication of aggressive surgical procedures (intestinal resections and anastomoses on an oedematous and swollen intestinal wall or stomas associated to intestinal stenosis and atresia) is the short intestine syndrome, the prognosis of which is guarded even in developed countries.

One of the problems we noted in our research is the fact that a quarter of the complex gastroschisis patients suffered from a short intestine syndrome after the surgical procedures, and only one of these six patients survived, since intestines are not transplanted in Romania. Also, since the hospital did not have proper equipment to measure the intra-abdominal pressure and adequate prosthetic material, the anterior abdominal wall was closed per primam under strain in 10 patients included in the study, nine of whom died due to the abdominal compartment syndrome. Perhaps these patients might have been saved if they had been treated by progressive integrations and/or by the application of an umbilical cord patch (which proved efficient when no other synthetic materials were available).

We suggest that Molik’s classification be changed to also include gastroschisis with liver herniation among the complex gastroschisis cases, due to the undeniable visceralabdominal disproportion, to the difficult integration of the protruding organs, sometimes to the associated extra-abdominal abnormalities and to the patients’ guarded prognosis.



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