Keywords

1 Gastroschisis

The patient usually has an abdominal wall defect at the right side of the umbilicus whose diameter is 2–3 cm. The prolapsed organs may include the intestine, stomach, reproductive organs, and bladder. The important points to keep in mind when we treat gastroschisis are as follows: (1) The size of the abdominal wall defect is usually not so large, (2) the intestine is a main prolapsed organ, and (3) intestinal problems such as prominent edema, atresia, and perforation are often seen.

1.1 Preoperative Management

In order to prevent severe dehydration and hypothermia, prolapsed organs should be covered by the Saran Wrap as soon as possible after birth. Also in order to protect the patient from bacterial infection, clean technique should be utilized to handle the patient using clean gauze, towels, and gloves.

1.2 Operations

1.2.1 The Case with the Prolapsed Organs Being Not Edematous (Fig. 27.1a)

When the prolapsed organs are not edematous, one-stage operation to close the abdominal wall defect should be tried. But if general condition and respiro-circulatory status are not stable, one-stage operation should be abandoned and a multistage operation be considered.

Fig. 27.1
figure 1

Gastroschisis (1). (a) A case with no edema in prolapsed intestine. (b) One-stage closure of the abdominal wall was performed in this case after the prolapsed intestine was pushed into the abdominal cavity. When the umbilical cord is preserved, the sutures to close the abdominal muscle cannot be stitched at the umbilicus, then the sutures are stitched near the umbilicus to adjust the left and right rectal muscles. (c) A case with prominent edema in prolapsed intestine. In this case, one-stage closure of the abdominal wall is difficult

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One-stage operation is usually performed under general anesthesia. The intestine should be carefully examined before reducing them into the abdominal cavity whether it has an atresia or a perforation. If the abdominal cavity is too small to reduce the prolapsed organs, the technique to distend the anterior abdominal wall using figure tip of the operator may be effective to gain enough cavity. Subcutaneous tissues around the abdominal wall defect are dissected well and abdominal muscular layer around the defect should be disclosed. The umbilical cord is preserved in situ in this stage to make the umbilical hollow. 3-0 absorbable sutures or nonabsorbable sutures are used to close the abdominal defect. The sutures cannot be stitched on the umbilical cord; instead the intimate sutures are stitched near the cord not to leave the space in the suture line (Fig. 27.1b). Skin closure is archived by the purse-string suture to create the circular umbilical scar.

1.2.2 The Case with Severe Edematous Intestine (Fig. 27.1c)

In some case, the intestine and other prolapsed organs have long been exposed in amniotic fluid in fetal life, which causes very severe inflammation and edematous change of the organs. And furthermore the abdominal wall defect is so small that the prolapsed organs are sometimes squeezed by the edge of the abdominal wall and it causes severe congestion and resultant edema. In these cases, multistage abdominal closure should be considered. One-stage operation is to construct the abdominal silo by the wound retractor AlexisR XS or XXS (Applied Medical Co. USA) (Fig. 27.2a). If the size of the abdominal wall defect is too small to create the silo in case the intestinal edema is very severe, we do not hesitate to enlarge the defect size to double by cutting the cranial side of the abdominal wall.

Fig. 27.2
figure 2

Gastroschisis (2). (a) In this case, the wound retractor AlexisR XS was inserted into the abdominal cavity to make a silo. The general condition in this case was poor and one-stage repair was abandoned. Outer side of the wound retractor is closed by the ligation. (b) The schema of the silo formation using wound retractor AlexisR XS. (c) The silo was squeezed day by day for a few days and the prolapsed intestine was put into the abdominal cavity. (d) After the abdominal muscle layer is closed, the skin defect is closed by the purse-string suture to form the umbilical pit

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When keeping the prolapsed organs in the bag, the blue ring of the wound retractor was inserted into the abdominal cavity after cutting a falciform ligament and a round ligament. The opened side of the bag is closed by rotating the white rings and ligated by the suture material (Fig. 27.2a, b). The skin edge around the defective abdominal wall should be covered by the appropriate material (we usually use Karayahesive® (Alcare Co. Tokyo Japan) to prevent the bacterial infection).

After the silo creation, the edema of the intestine gradually subsides by the improvement of the blood flow and the prolapsed intestine starts to put into the abdominal cavity naturally. The operator squeezes the bag so as to push the prolapsed organs into the abdominal cavity once a day. Within 7–10 days, prolapsed organs are put into the cavity. Intestinal ischemia and the excessive increase of the intra-abdominal pressure must be avoided during these procedures by careful monitoring. Under general anesthesia, the final operation to close the abdominal wall defect should be performed (Fig. 27.2c). Remove the wound retractor and close the abdominal muscle layer according to the same way described in the one-stage operation. Skin closure is also done by the purse-string suture. After the operation, respiratory support is usually needed for a few days (Fig. 27.2d).

1.2.3 The Case Being Complicated with Intestinal Atresia or Intestinal Perforation

If the intestinal atresia is apparent at birth, intestinal stoma is created first, and after decompressing the intestinal distension, primary closure or multistage closure of the abdominal wall defect should be selected. If the intestinal perforation exists and abdominal cavity is contaminated with meconium, silo creation should be selected at birth with appropriate drainage of the abdominal cavity.

1.2.4 Sutureless Method to Close the Abdominal Wall Defect (Fig. 27.3)

This method has attracted attention because of its easy procedure. Instead of abdominal wall closure by suture materials, the skin edge is covered by Tegaderm™ and waits to close the muscle layer by itself.

Fig. 27.3
figure 3

Sutureless closure of the abdominal wall. In this case after the prolapsed intestine was put into the abdominal cavity, the skin defect is closed by the tape fixed on the skin without using any suture materials. Abdominal wall close naturally in this method

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2 Omphalocele

Omphalocele is often complicated with other congenital anomalies such as cardiac disease or chromosomal anomaly, which sometimes define the prognosis of the patients. The classification of omphalocele is as follows: (1) supraumbilical, (2) umbilical, (3) infraumbilical, and (4) umbilical cord hernia.

2.1 Preoperative Management

Almost the same as gastroschisis

2.2 Operations

2.2.1 Umbilical Cord Hernia

Some bowels prolapsed into the umbilical cord. This type of hernia is less severe and primary closure is easy. It is recommended that the primary closure should be done under general anesthesia. First hernia sac should be removed and check whether some congenital anomalies such as omphalomesenteric remnants or urachal remnants exist. The prolapsed bowels are easily reduced into the abdominal cavity and close the abdominal wall by a few sutures.

2.2.2 Giant Hernia Containing Liver Parenchyma (Figs. 27.4 and 27.5)

Fig. 27.4
figure 4

Omphalocele (1). A case of moderate-size omphalocele (hernia content includes only the liver). (a) Cyst formation in the Wharton jelly is ascertained. (b) Wound retractor AlexisR XS is inserted into the abdominal cavity after removing the hernia sac. A case of giant omphalocele (hernia contents include the liver and intestine). (c) This hernia is very large in size and anticipated much difficulty to put the herniated organs into the abdominal cavity. (d) Wound retractor AlexisR XS was directly sutured to the edge of the defected skin to form a silo

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Fig. 27.5
figure 5

Omphalocele (2). (a) A case presented in Fig. 27.4c, d. In 7 days, the almost all prolapsed organs were reduced into the abdominal cavity by squeezing the silo day by day. (b) The abdominal wall is closed by the muscular layer after the hernia sac was removed. The muscle layer is stretched with the strong tension in this case. (c) The skin defect is longitudinally sutured and the skin at the caudal end is plicated as rectangular shape to form a skin roll that is imitated as an umbilical pit

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  1. (a)

    After dissecting widely, the subcutaneous tissue around the hernia sac and abdominal wall defect is covered with freed skin. Defective abdominal muscle is left open and it will be closed later in life. This method is now rarely adopted in Japan.

  2. (b)

    Silo formation by wound retractor AlexisR.

    One method is a direct suture of a retractor ring to abdominal muscle layer after dissecting subcutaneous tissues around the hernia. This method effectively pulls up the abdominal muscle and dilates the abdominal cavity but may cause the injury of the muscle layer by sutures when squeezing the prolapsed organs into the abdominal cavity.

    Another method is almost the same way as gastroschisis. This method should be applied to the omphalocele with relatively mild liver prolapsed (Fig. 27.4a). After removing the hernia sac, the blue ring of the wound retractor is inserted into the abdominal cavity (Fig. 27.4b). This method is less effective to dilate the abdominal cavity and the ring may be slipped out by the tension.

    When the hernia is large and abdominal wall is so hypoplastic (Fig. 27.4c), the blue ring of the wound retractor is directly sutured to the skin edge of the hernia leaving hernia sac in situ (Fig. 27.4d). This method may be possible under local anesthesia. As hernia sac is left intact, ascites does not overflow from the wound and it is easy to squeeze the prolapsed organs into abdominal cavity. In 7–10 days, the prolapsed organs should be pushed into the abdominal cavity because the skins will be torn by the sutures with the tensions (Fig. 27.5a). If the hernia sac adheres to intra-abdominal organs, hernia sac is partly resected and the adhesion is released before constructing the silo. The abdominal wall is closed by muscular layer after the prolapsed organs are reduced into the abdominal cavity by silo squeezing (Fig. 27.5b). In this method, the hernia sac is intact in situ and we first dissect the hernia sac from the skin and the abdominal muscle is closed by the absorbable sutures. The skin cannot be closed as the gastroschisis because the skin defect is large enough to close by purse-string suture. We close the skin longitudinally and at the caudal end, the skin is cut rectangle and make a skin roll to form the umbilical pit (Fig. 27.5c).

  3. (c)

    Recently survival cases of a more complicated and severe omphalocele have been reported because they are well cared in fetus and intensively cared after birth with the up-to-date respiratory support. In such severe cases, the defect of the abdominal wall is so severe and the development of abdominal muscle is so poor. In order to close the abdominal wall, the skin graft is applied or KarayahesiveR (Alcare Co. Tokyo, Japan) is applied on the hernia sac to induce skin elongation to cover the defect. In some case, component separation technique is applied to close the abdominal muscle: the fascia of the external oblique muscle is longitudinally cut and it makes the transverse widening of the muscle to gain enough abdominal cavity. In another case, the tissue expander is inserted into the abdominal cavity to make enough space to reduce the prolapsed organs.