Discussion
Pancreatic pseudocysts are a rare but potentially troublesome problem in children. They can occur as the result of pancreatic insult with ductal disruption, often secondary to trauma. Pseudocyst drainage is warranted when attempts at conservative management fail. Current approaches to draining pancreatic pseudocysts include open or laparoscopic surgery, endoscopic drainage, and image-guided percutaneous internal or external drainage.
Surgical drainage has been the traditional method [
8]. This is traditionally performed by an open approach, and more recently by laparoscopic techniques that attempt to replicate their open equivalents [
9]. Surgical techniques include formation of a cystogastrostomy if the pseudocyst is adherent to the posterior wall of the stomach, or otherwise formation of a Roux-en-Y cystojejunostomy [
9]. Surgery is particularly useful in people with unfavourable anatomy [
9].
Endoscopic drainage of pseudocysts was introduced in the mid-1980s and is reported to be safe and effective. It has since become the primary therapeutic modality for drainage of pseudocysts [
1,
3]. The use of endoscopic ultrasound has evolved more recently [
10,
11]. Endoscopic ultrasound can help in finding the optimal site for puncture of the pseudocyst and subsequent stent placement by assessing wall thickness and the distance from stomach or duodenum to pseudocyst, and by identifying major vascular structures [
3,
10,
11]. However endoscopic ultrasound has been limited mostly to diagnostic use in children, with only a few case reports describing its therapeutic role in pseudocysts [
3].
Endoscopic drainage can be performed from transpapillary or transmural approaches. Endoscopic transmural drainage is usually performed with endoscopic US guidance. Endoscopic and endoscopic US-guided transmural drainage require direct apposition with visible bulging of the gastric or duodenal wall at the site of the pseudocyst and a cyst wall thickness less than 1 cm [
1,
9,
12], and drainage is usually achieved by placement of one or two plastic stents or a metal stent.
Percutaneous drainage can be performed with US or CT guidance and can be achieved by a retroperitoneal or transperitoneal route [
9]. Percutaneous drainage is now reserved for unstable patients, immature cysts, and patients with infected pancreatic pseudocysts [
13]. It is infrequently performed because of the risk of creating a cystocutaneous fistula.
Percutaneous cystogastrostomy formation has also been reported in adults [
7,
14,
15]. This has been done using double pigtail catheters with US and fluoroscopic guidance [
7,
14]. Initially this was a two-stage procedure involving placement of a stent between the stomach and pseudocyst after 7 days [
15], but more recently single-step procedures have been reported [
7]. In adult series of up to 12 patients percutaneous cystogastrostomy has been shown to have a high success rate with good short-term outcomes [
7]. This technique avoids the incisions of up to 2 cm that are made in the gastric wall with a knife during endoscopic drainage and can cause bleeding or perforation of the peritoneum [
7].
There is debate regarding the approaches to treatment of pancreatic pseudocysts even in adults, in whom they occur much more frequently than in children. A systematic review comparing the outcomes of endoscopic, percutaneous and surgical pancreatic pseudocyst drainage included results from 10 comparative studies, of which three were randomised controlled trials [
9]. It found no consensus about the best management approach [
9]. Endoscopic US-guided drainage appeared to be advantageous for pancreatic pseudocysts located adjacent to the stomach or duodenum. Surgical cystojejunostomy or percutaneous drainage can be considered for people with unfavourable anatomy [
9]. There have been reports of the successful use of all of these approaches in children but, as in adults, there is no consensus regarding best treatment. Our series of six children, in which technical success was 100%, demonstrates that cystogastrostomy using interventional radiology techniques is feasible in children and therefore might be a useful alternative to surgery or interventional endoscopic ultrasound. The recurrence rate after surgery has been reported to be as high as 10% [
1]. This series included one child who had re-accumulation after a previous attempt at surgical drainage, but there were no pancreatic pseudocyst re-accumulations in our patients after image-guided cystogastric stent insertion.
It has been recommended that all cystoenterostomies be stented to avoid recurrence [
10]. Often two double-pigtail plastic stents are placed to maintain patency, allow complete resolution of the pseudocyst and minimise the effect of spontaneous stent migration [
10], but these often need replacement because of dysfunction and migration [
13]. Further, endoscopic placement of multiple plastic stents can be technically difficult, so the use of a single completely covered self-expanding metallic stent has been proposed as an alternative [
13]. These devices allow a single-step creation of a large-diameter fistula [
13]. In a number of studies, including a recent meta-analysis, of metal versus plastic stents for drainage of pancreatic fluid collections, the use of metal stents was found to be associated with improved clinical success, fewer adverse events and reduced bleeding compared to plastic stents [
16,
17]. However stent migration occurs in up to 15% of patients [
13] and bleeding requiring embolisation, stents becoming buried under gastric mucosa, and a biliary stricture related to mechanical compression from a stent have all been reported [
18]. Additionally, even in adults, there is a paucity of long-term safety data.
The self-expanding covered nitinol pancreatic pseudocyst drainage stent used in this case series (Niti-S Nagi), although designed for endoscopic placement, can be placed using image guidance and a transgastric approach. The advantage of this device is that the large (20-mm) acute-angled flared ends anchor the stent in the stomach and pseudocyst and help prevent migration [
13]. It should be noted that according to information from the manufacturer, a stent might require 1–3 days to expand fully and a soft diet is suggested after insertion. This stent has been reported to have been used for endoscopic US-guided drainage in 21 children (mean age 14.9 years) with technical and clinical success rates of 100% and 95%, and no major complications [
19]. However stent migration was noted in 1 child [
19], as in our series, and occurred in 4 of 21 adults in another series [
20]. Another advantage of this device is the ease of deployment. Other image-guided cystogastrostomy series have used biliary endoscopic stents designed to treat calculi (overall length 10 cm) and have reported that they are too long and therefore difficult to deploy when the stomach lumen is reduced by the mass effect of the cyst [
7]. The stents used in this study are available in lengths of 10–30 mm and therefore do not cause this problem. Another advantage of the stent is that it is of sufficient size to allow endoscopic necrosectomy should this become required.
It is recommended that flanged self-expanding covered nitinol pancreatic pseudocyst drainage stents be removed after the confirmation of complete resolution of the pseudocyst [
20]. The stents should be removed endoscopically by grasping the retrieval string with forceps and collapsing the proximal end of the stent with a snare and then carefully pulling. In a study in which an attempt to remove a flanged self-expanding covered nitinol pancreatic pseudocyst drainage stent was made in 16 people, all stents were easily removed with no complications 12 months after placement [
20].
Complications of percutaneous image-guided drainage appear to be uncommon. In a study of 12 people who underwent image-guided cystogastrostomy, there was post-procedural sepsis in two people with ongoing pancreatitis and only partial drainage in another [
7]. In our series of six children there were no complications as a result of intervention. Reported complications of endoscopic drainage of pancreatic pseudocyst include bleeding, intestinal perforation, infection, leakage, stent migration and pseudocyst recurrence [
1]. Endoscopic drainage has fewer complications than surgical drainage, which has been shown in adults to have a morbidity of up to 35% and mortality of up to 10% [
3,
21].
In this series a pre-existing gastrostomy tract was used to access the stomach in two children. In such cases, the stomach is already adherent to the anterior abdominal wall and a new stomach puncture does not have to be performed. Four children in this study required a gastrostomy to be formed as part of the drainage procedure. Complications of gastrostomy formation by interventional radiology are reported to be 0–5% including peritonitis in up to 3% of cases, other infective complications such as subcutaneous abscess in 2% and septicaemia in 1%, and bowel transgression in 0.2% of cases [
22]. Insufflation of air to facilitate stomach puncture can obscure subsequent pseudocyst visualisation by US and should be avoided if possible. If necessary, insufflation of the stomach with saline is a better alternative that allows for continued sonographic visualisation of the pseudocyst. Gastropexy sutures were not used in two of the four children in which there was a new stomach puncture. These children did not develop complications, suggesting the hole created in the anterior surface by the up to 12-Fr dilator closed without significant peritoneal soiling.
Pancreatico-cutaneous fistula can occur after drainage of pseudocysts and can be very difficult to treat. The risk of fistula formation might be reduced by performing a single-step procedure because the gastrostomy created is allowed to heal [
7]. Avoiding the use of gastropexy devices might further reduce this risk. External drainage is usually avoided in adults because of a high incidence of pancreatico-cutaneous fistula formation, although this risk is lower in children [
23].
This retrospective study has some limitations that must be acknowledged. The number of children included is small; however this reflects the rarity of the condition. Also, the precise technique including the use of prophylactic antibiotics, the tract dilation method and the use of gastropexy sutures varied slightly among patients. The generalisability of the technique to other paediatric hospitals is unknown. Endoscopic US-guided drainage has become common in adults but its application in children is lagging [
3]. This is presumably because of the lack of experience in paediatric hospitals related to the rarity of this condition [
3]. In small children, endoscopic US drainage can be technically difficult because of the large size of the US endoscope [
19]. The requirement for specialised equipment might also limit the use of endoscopic US-guided drainage in children. Adopting a radiologic approach to drainage might have similar drawbacks. Training in low-volume, high-complexity procedures is a known problem in paediatric interventional radiology [
24]. Paediatric interventional radiology procedures involve a set of core skills, however, involving access to the appropriate site under image guidance (often using a needle or catheter and guidewire) followed by some type of intervention [
24], so most paediatric interventional radiologists could undertake pancreatic pseudocyst drainage.
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