*2.5.4 Surgical treatment*

In uncomplicated meconium ileus, surgical exploration is indicated when there is progressive distention or signs of clinical deterioration or peritonitis despite multiple enemas. Whereas in complicated cases (e.g., meconium peritonitis, ileal atresia or stenosis, ileal perforation, and volvulus with or without pseudocyst formation), surgery is always indicated.

Indications for surgical management in meconium ileus [31]:


In the operative management of simple uncomplicated meconium ileus, the aim is to evacuate meconium from the intestine without resecting any bowel segment; however, this might be inevitable in certain instances. On the other hand, complicated meconium ileus requires resection more often and may necessitate the use of temporary stomas.

The fibrous wall of the pseudocyst is debrided without sacrificing viable intestine. Extensive adhesiolysis is required for adhesive obstruction due to meconium peritonitis; these adhesions are typically dense and very vascular. It is not necessary to perform a radical debridement of all meconium calcified plaque encountered, as long as the obstruction is relieved [32].

The surgical approach for treatment of uncomplicated meconium ileus should be individualized for each infant, although many procedures have been proposed over the years with variable success rates achieved. In all cases, uncomplicated or complicated, the following procedures are commonly done:


#### *2.5.5 Enterotomy and decompression*

An enterotomy is made on the antimesenteric border of the dilated ileum for instillation of irrigation solution (dilute acetylcysteine or saline solution) which help to loosen the inspissated meconium and liquefy it for effective evacuation through the enterotomy. The irrigation solution is introduced using a size 10 French catheter, and both the proximal and distal loops of bowel are irrigated. After complete decompression of inspissated meconium, the enterotomy is closed transversely. An appendectomy is performed with the specimen sent for histologic examination to detect the presence of ganglion cells, as well as possible presence of mucous plugging of the crypts and exuberant intraluminal mucinous material, which are suggestive of cystic fibrosis. Postoperatively, a gentle anal dilatation and rectal irrigation may sometimes be required for further evacuation of large amount of meconium passed distally into the colon during intraoperative irrigation. Enterotomy and decompression are usually indicated for simple uncomplicated meconium ileus. A supraumbilical transverse incision or transverse right lower abdominal incision can be used for the procedure.

#### *2.5.6 Enterostomy with subsequent irrigation*

In cases where the irrigation done after an enterotomy cannot effectively evacuate the inspissated meconium despite a patient approach, an indwelling ostomy tube (e.g., T-tube) can be inserted for postoperative bowel irrigation decompression. The irrigations are started on the first postoperative day and continued for 7–14 days. After successfully evacuating the inspissated meconium, the tube is removed, and the enterocutaneous fistula thus formed is allowed to close spontaneously. T-tube enterostomy was first described by Harberg et al. in 1981 [33]. Enterostomy for postoperative irrigation can also be done without using tube; in which case the ileal opening is sutured to skin and the bowel tacked to the fascia in standard fashion.

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**Figure 2.**

*Schematic description of some enterostomies as copied from Ref. [36].*

*Meconium Ileus*

*DOI: http://dx.doi.org/10.5772/intechopen.85548*

*2.5.7 Resection with enterostomy or anastomosis*

continuity [25, 31]. Hence, they are rarely used today.

Bowel resection is indicated when meconium ileus is associated with a nonviable bowel, bowel perforation, atresia, volvulus, and the like. Resection is usually combined with enterostomy procedure, but primary anastomosis may be done if the intraoperative findings and the patient's general condition are favorable. The disadvantages of the procedures involving resection and stoma(s) or anastomosis are potential postoperative fluid losses through high-volume stomas, bowel shortening by resection, and the need for a second procedure to reestablish intestinal

Various stoma operations have been described with the most widespread being the Bishop-Koop-type anastomosis. This is a Roux-en-Y construct in which the distal limb is brought out as an end stoma and the proximal bowel is anastomosed end-toside approximately 4 cm from the opening of the distal segment (**Figure 2**). Normal gastrointestinal transit is permitted by this technique, and should distal obstruction

The reverse of the Bishop-Koop enterostomy is the proximal enterostomy, described

The Mikulicz enterostomy, first reported by Gross in 1953, consists of a doublebarrel stoma in which the two ends are sutured together side to side for some length

occur, it provides a means for management through the ileostomy [25, 31].

technique predisposes to high-output losses with inherent risk of dehydration.

by Santulli and Blanc in 1961 [25, 31]. In this technique, the end of the distal limb is anastomosed to the side of the proximal limb after resection, while the end of the proximal limb is brought out as the enterostomy (**Figure 2**). This arrangement enhances proximal irrigation and decompression, thus making intraoperative evacuation of the dilated proximal bowel loop unnecessary. A catheter can be inserted into the distal limb through the stoma for irrigation of the distal bowel. The proximal stoma created in this

*Pediatric Surgery, Flowcharts and Clinical Algorithms*

long as the obstruction is relieved [32].

• Enterotomy and decompression

• Resection and enterostomy

• Resection and anastomosis

*2.5.5 Enterotomy and decompression*

abdominal incision can be used for the procedure.

*2.5.6 Enterostomy with subsequent irrigation*

to the fascia in standard fashion.

complicated, the following procedures are commonly done:

• Enterostomy (with or without tube) with subsequent irrigation

temporary stomas.

In the operative management of simple uncomplicated meconium ileus, the aim is to evacuate meconium from the intestine without resecting any bowel segment; however, this might be inevitable in certain instances. On the other hand, complicated meconium ileus requires resection more often and may necessitate the use of

The fibrous wall of the pseudocyst is debrided without sacrificing viable intestine. Extensive adhesiolysis is required for adhesive obstruction due to meconium peritonitis; these adhesions are typically dense and very vascular. It is not necessary to perform a radical debridement of all meconium calcified plaque encountered, as

The surgical approach for treatment of uncomplicated meconium ileus should be individualized for each infant, although many procedures have been proposed over the years with variable success rates achieved. In all cases, uncomplicated or

An enterotomy is made on the antimesenteric border of the dilated ileum for instillation of irrigation solution (dilute acetylcysteine or saline solution) which help to loosen the inspissated meconium and liquefy it for effective evacuation through the enterotomy. The irrigation solution is introduced using a size 10 French catheter, and both the proximal and distal loops of bowel are irrigated. After complete decompression of inspissated meconium, the enterotomy is closed transversely. An appendectomy is performed with the specimen sent for histologic examination to detect the presence of ganglion cells, as well as possible presence of mucous plugging of the crypts and exuberant intraluminal mucinous material, which are suggestive of cystic fibrosis. Postoperatively, a gentle anal dilatation and rectal irrigation may sometimes be required for further evacuation of large amount of meconium passed distally into the colon during intraoperative irrigation. Enterotomy and decompression are usually indicated for simple uncomplicated meconium ileus. A supraumbilical transverse incision or transverse right lower

In cases where the irrigation done after an enterotomy cannot effectively evacuate the inspissated meconium despite a patient approach, an indwelling ostomy tube (e.g., T-tube) can be inserted for postoperative bowel irrigation decompression. The irrigations are started on the first postoperative day and continued for 7–14 days. After successfully evacuating the inspissated meconium, the tube is removed, and the enterocutaneous fistula thus formed is allowed to close spontaneously. T-tube enterostomy was first described by Harberg et al. in 1981 [33]. Enterostomy for postoperative irrigation can also be done without using tube; in which case the ileal opening is sutured to skin and the bowel tacked

**78**
