Fig. 12.1
Types of pancreatic and peripancreatic collections based on the revised Atlanta classification system of acute pancreatitis with representative contrast-enhanced CT images. Collections are differentiated by the presence or absence of necrotic debris, and their chronicity (greater than or less than 4 weeks). a Acute peripancreatic fluid collection (APFC; white arrows show border of APFC). b Pancreatic pseudocyst ( white arrows show border of pseudocyst). c Acute necrotic collection after an episode of severe acute necrotizing pancreatitis ( white arrows show border of collection). d Same patient as C, now a few weeks later with walled-off pancreatic necrosis (WOPN; white arrows show border of WOPN, black arrowheads show areas of debris)
Pancreatic and peripancreatic collections manifest as a variety of symptoms and complications based on the location and extent of the fluid collection. The presence of a collection is often associated with abdominal pain of variable intensity, distention, and anorexia. These symptoms are not relieved as the other manifestations of pancreatitis resolve. In addition, based on the location of the collection, local duodenal or biliary obstruction may be seen and may need to be addressed separately. A fistula can also develop to surrounding structures, which can present as drainage from a pancreaticocutaneous fistula, ascites from a pancreaticoperitoneal fistula [5] or shortness of breath from a pancreaticopleural fistula [6].
Distention from a fluid collection can be severe and may lead to more diffuse abdominal compartment syndrome [7]. Abdominal compartment syndrome (ACS) is a sustained elevation of the intra-abdominal pressure (IAP) that is associated with new onset organ failure or acute worsening of existing organ failure. It typically presents as a tensely dilated abdomen, oliguria, and increased peak airway pressures. In necrotizing pancreatitis, it appears early and is thought to be related to a combination of mass effect from a necrotic collection, acute inflammation, edema of surrounding tissue, and possibly over resuscitation. The diagnosis is made by measuring bladder pressure with a transurethral probe [8]. Normal bladder pressures in hospitalized patients are 5–7 mmHg, with decreased perfusions developing at 12 mmHg or more. Organ failure and thus ACS begins to develop at bladder pressures >20 mmHg. Identification of ACS is critical as it can be associated with up to 50 % mortality and 90 % morbidity [7]. Initial management consists of sedation , neuromuscular blockade, nasogastric decompression, and correction of a positive cumulative fluid balance that may be contributing to increased IAP. If these fail, percutaneous catheter drainage of an acute fluid collection or peritoneal fluid may be necessary. Finally, if these fail, surgical decompression can be performed and an open abdomen maintained.
Peripancreatic collections can also result in vascular pathology. Erosion of pancreatic fluid into surrounding vasculature may result in a pseudoaneurysm . If bleeding occurs from a pseudoaneurysm into a collection, it will likely present as worsening or severe abdominal pain. If bleeding occurs into the pancreatic duct, it will manifest as hemosuccus pancreaticus (Chap. 16) with intermittent melena and potentially abdominal pain. Imaging with either contrast-enhanced computed tomography angiography (CTA), magnetic resonance angiography (MRA), or sometimes with MRCP alone is used to identify pseudoaneurysms. These should be managed separately by interventional radiologists [9], and by surgery in the setting of chronic pancreatitis. If there is pancreatic necrosis, vascular involvement may also present as thrombosis of the splenic, portal, or superior mesenteric veins. These can result in development of collateral veins and varices, which may be seen during endoscopic evaluation (see walled-off pancreatic necrosis below). Direct complications from such a thrombosis are rare and treatment is rarely required [10] unless there are significant varices, extensions to the inferior vena cava or impending renal compromise.
Case 1
A 37-year-old male with a prior history of diabetes and high cholesterol presented with severe acute abdominal pain radiating to his back. On initial examination, he was hypotensive and tachycardic. He was alert, but slightly tachypneic and had a distended abdomen with diminished bowel sounds. On initial workup, his lipase was 1840 U/L (normal 13–60 U/L). Immediate fluid resuscitation with Lactated Ringer’s (LR) was begun. Further workup showed that his liver function tests and right upper quadrant ultrasound were normal, and per his family, there was no history of heavy alcohol use. Further laboratory tests showed a triglycerides level of 2000 mg/dL. He was admitted to the ICU, continued on 300 mL/h LR to maintain his urine output at 0.5 mL/kg/h, and started on appropriate insulin therapy. Bladder pressures remained 10–12 mmHg and slowly his hemodynamics and laboratory studies normalized over the next few days. Unfortunately, he continued to be in pain and his abdomen remained distended. A contrast-enhanced computed tomography (CT) of his abdomen and pelvis showed an edematous pancreas without definitive necrosis and a large 17 cm × 12 cm × 18 cm homogeneous cystic collection with minimal rim enhancement anterior to the pancreas (Fig. 12.2). No significant debris was seen within the collection. Surgery and gastroenterology were consulted for the management of the fluid collection and continued abdominal pain.
Fig. 12.2
Contrast-enhanced CT scan of the abdomen from Case 1—a 37-year-old male with acute pancreatitis thought secondary to hypertriglyceridemia. A large acute peripancreatic collection was found as shown on a axial and b coronal CT. Arrows show border of collection
What Are Our Initial Diagnostic and Therapeutic Options?
The patient in the case illustrated has a large collection that has not been fully characterized. Once a pancreatic or peripancreatic fluid collection is suspected, imaging to better classify its location and the type of collection is important. In particular, the identification of necrotic debris in a collection can be crucial in clinical management, as this would require endoscopic debridement instead of endoscopic drainage alone. The modality most commonly used is a contrast-enhanced CT (Table 12.1) [2]. CT can identify the extent of the collection and the presence of necrosis, although the sensitivity and specificity can be low [11]. Studies have shown that magnetic resonance imaging (MRI) can have higher sensitivity and specificity in evaluating necrotic debris and may be more helpful than CT in indeterminate cases [12, 13]. MRI has the advantage of avoiding exposure to harmful radiation, which can be significant in a younger patient requiring multiple imaging studies [14].
Table 12.1
Morphological features of the four types of pancreatic and peripancreatic collections typically seen on radiological imaging
Associated type of pancreatitis | Density on CT | Encapsulated | Location | |
---|---|---|---|---|
APFC | Interstitial edematous | Liquid | No | Extrapancreatic |
Pseudocyst | Interstitial edematous | Liquid | Yes | Extrapancreatic |
ANC | Necrotizing | Liquid and non-liquid | No | Extra- and/or intrapancreatic |
WOPN | Necrotizing | Liquid and non-liquid | Yes | Extra- and/or intrapancreatic |
Cholangiopancreatography protocol with MRCP can be used to detect pancreatic ductal disruptions and pancreatic fistulae. MRCP can be further enhanced with the administration of secretin, a hormone that induces pancreatic ductal secretion resulting in better visualization of the pancreatic duct morphology and any fistulae. Recent data have shown that the sensitivity of detecting pancreatic ductal anomalies can be increased from 47 to 66 % by performing MRCP with the administration of secretin [15, 16]. However, secretin-MRCP is not currently widely adopted.
It should be noted that pancreatic cystic neoplasm is an important differential diagnosis that must be excluded. The management strategy will change significantly if the fluid collection is a cystic neoplasm as the patient may need to undergo surgery. Clinical history, as well as comparison with any available abdominal radiology before the pancreatitis attack, is fairly reliable in making this distinction. In rare cases, if there is confusion regarding the etiology of the collection, endoscopic ultrasound (EUS) or percutaneous sampling may be necessary.
Initial Management of a Collection
Initial steps in the management of acute pancreatitis consist of aggressive supportive care including IV hydration with lactated ringer’s, pain management, and efficient steps to identify the cause of pancreatitis . Once a collection has been characterized, the complications of the collection may need to be addressed separately (see Introduction). If after the management of the acute pancreatitis and these complications , no or minimal symptoms remain, conservative management of the collection with watchful waiting can be attempted. Data on pancreatic collections show that in patients that can be managed without intervention, spontaneous resolution can occur in 30–60 % of patients [17–19]. More specific data on the different types of acute fluid collections are sparse, although based on a few studies, we know that fluid collections associated with non-necrotizing pancreatitis resolve faster, often within 2 weeks in about 70 % of patients [20] compared to spontaneous resolution in about 30 % by month 6 in patients with necrotizing pancreatitis [17].
Back to the Case…
The patient’s pain and symptoms were managed for the next 2 weeks as he improved, although he continued to have baseline abdominal pain, distention, and anorexia. Therefore, MRI of the pancreas with MRCP was obtained, which showed the large abdominal fluid collection had grown slightly, again without any evidence of necrotic debris. In addition, the fluid collection was likely communicating with the main pancreatic duct in the mid-body, which probably represented a pancreatic duct disruption.
Transpapillary Pancreatic Duct Stenting
Timing and Approach
In the case detailed above, a pancreatic duct disruption has occurred, which is likely resulting in the development of an acute peripancreatic fluid collection. Duct disruption regardless of etiology (acute pancreatitis, pancreatic surgery, or trauma) can be treated using a similar endoscopic approach. Initial conservative therapy for ductal disruptions can include nasojejunal feeding, somatostatin analogues, and pancreatic enzyme replacement. Nasojejunal feeding is associated with significantly higher spontaneous closure rate for post-surgical pancreatic duct fistulae, presumably by reducing pancreatic stimulation [21]. Somatostatin analogues are routinely used perioperatively for pancreatic surgeries [22, 23] although there are only a few studies describing its use for pseudocysts and the practice remains controversial [24].
If conservative management has not resulted in any improvement, either clinically or on imaging (as with our patient), ERCP with transpapillary pancreatic duct stent placement should be attempted [3, 5, 25, 26]. One scenario where transpapillary stenting is of unclear utility is the disconnected duct syndrome [5, 27]. This involves full transection of the pancreatic duct, and the proximal (tail) portion of the pancreas freely secretes pancreatic juices. On pancreatogram, there is either blowout of the duct or a complete cutoff with no duct opacified upstream from this area (Fig. 12.3). Given the full transection, a bridging stent cannot be placed and management focuses on creating a cystgastrostomy to allow a fistulous connection back to the lumen of the stomach or bowel. If this approach fails, combined percutaneous and endoscopic rendezvous procedures may succeed [27], and surgical options include pancreaticojejunostomy and distal pancreatectomy. Percutaneous drainage is not an appealing option as the rate of persistent external pancreatic fistula from the drains is high [27].
Fig. 12.3
ERCP fluoroscopy images of disconnected duct syndrome after acute pancreatitis. The pancreatogram demonstrates a complete pancreatic duct disruption with contrast leaking out in area of the neck and no contrast filling the upstream pancreatic duct. Bridging stent placement is not possible and treatment focuses on creation of a cystgastrostomy
Timing for ERCP can vary significantly based on the clinical scenario, from immediately on recognition of the ductal leak to >4 weeks after the episode of acute pancreatitis . On the basis of the literature, we generally try to wait 4–6 weeks for patients with pancreatitis-related duct disruption, although patient’s discomfort or more acute symptoms may lead us to intervene sooner. ERCP is performed in a standard fashion, and the site of disruption is identified during pancreatography. In addition, the location of any stricture or stones should be noted. Most often, a 5Fr or 7Fr stent is utilized, although the exact size will depend on the clinical scenario and has not been shown to be related to successful closure of the PD leak. Any strictures or stones that may have contributed to the leak should be traversed and managed. In addition, the stent should ideally bridge the disruption to maximize chances of success (see next section). The stent should be left in place at least 6 weeks, although time to closure can vary significantly with studies reporting a median closure time as high as 4 months [3, 26]. Prophylactic antibiotics should be administered with all these procedures since ERCP introduces non-sterile contrast into an otherwise sterile fluid collection.
It should be noted that depending on the clinical scenario (including the degree of symptoms from the fluid collection and maturity of wall around the pseudocyst), many investigators have reported performing a cystgastrostomy in the same endoscopic session as the ERCP, particularly for large encapsulated fluid collections. Hookey at al [28] in a retrospective chart review and prospective follow-up, reported on the resolution of pancreatic fluid collections in 15 patients with transpapillary stent alone, 60 patients with transmural drainage alone, and a combined approach in 41 patients. Overall clinical success (resolution of symptoms and collection) was 88 % and was not dependent on the drainage technique performed. However, patient characteristics differed significantly among the different groups. For example, the 15 patients with transpapillary stenting alone typically had a smaller fluid collection (<7 cm) with evidence of pancreatic duct obstruction and communication between the pancreatic duct and the pseudocyst. A combined approach was used for larger cysts communicating with the pancreatic duct or when the transpapillary approach was unable to bridge the leak. These data contrast with a small study from Singh’s group in India, who employed a pure transpapillary approach for larger cysts (> 7 cm) in the tail of the pancreas and found resolution in only 33 % of patients, while the other 67 % were complicated by infection requiring further percutaneous drainage. Overall, transpapillary stenting alone is not recommended for large mature fluid collections where transmural drainage or combined transmural drainage and transpapillary stenting likely has the best outcome. Immature fluid collections cannot be drained transmurally, and would require transpapillary stenting if drainage is necessary.
Outcomes and Alternative Treatment of Failures
ERCP with transpapillary stenting is fairly effective for the treatment of pancreatic duct leaks [3, 26, 29, 30]. Review of literature shows resolution of pancreatic duct leaks in 58–100 % of patients, although the etiology of the pancreatic duct leak in these studies included cases from chronic pancreatitis, trauma, and post-surgical leaks in addition to acute pancreatitis . In addition, the technique varied substantially and may explain the variability of the outcomes. Success was positively associated with findings of a partial duct disruption, location of the disruption in the body of the pancreas, maintaining the stents for at least 6 weeks, and placement of stent that bridges the duct leak. Data from our institution [3] showed that a stent that bridged a disruption was associated with successful resolution of a leak in 92 % of patients. Patients in whom the stent was placed only across the papilla or up to the disruption, by comparison, were associated with only a 44–50 % success rate (Fig. 12.4), stressing the importance of bridging a disruption.
Fig. 12.4
a A stent bridging a pancreatic duct (PD) disruption is associated with a 92 % resolution rate, versus b 50 % for a stent that ends at the leak and c 44 % for a stent that only crosses the papilla. ( Adapted from [3])
Mortality of this procedure is rare and complications (7–9 %) are mainly associated with performance of the ERCP. More specific complications include fevers and infection following stent placement, stent occlusion, or transpapillary stenting alone of large pseudocysts. Recurrence in the setting of stent failure or stent dislodgement can be treated with repeat ERCP and restenting for another 6–8 weeks. With unsuccessful procedures occurring from either failure to place the pancreatic duct stent or failure to resolve the leak, a trial of draining the fluid collection alone can be attempted (see below, Endoscopic Ultrasound and Transmural Drainage of a Pseudocyst). About 4 % ultimately require surgery; surgical options include a pancreaticojejunostomy and a distal pancreatic resection for ductal disruption in the body and tail. Overall success of the surgical approach is high at 90–92 %, although with a mortality of 6–9 % [31].
Case Continued
The patient was taken to the endoscopy suite and the site of the leak was readily identified during pancreatography. A 7Fr x 9 cm plastic stent was placed in the patient’s pancreatic duct, bridging the pancreatic duct leak (Fig. 12.5). Cystgastrostomy could not be performed as the wall around the pseudocyst had not matured yet. The patient did well immediately post-procedure with gradual decline in his abdominal pain. However, the pain did not resolve and he continued to endorse anorexia and gradual weight loss. Repeat contrast-enhanced CT four weeks after the ERCP and stent placement showed an 8 cm x 10 cm fluid collection adjacent to body of the stomach, now with a well-formed wall.
Fig. 12.5
ERCP representative of Case 1. a Pancreatic duct leak noted on ERCP with pancreatogram showing partial disruption in the body of the pancreas with contrast leaking out around the duct. b Plastic stent placed bridging the leak
Endoscopic Ultrasound and Transmural Drainage of Pseudocysts
Technique of Endoscopic Cystgastrostomy
Later sequelae of a pancreatic duct leak can include pancreatic ascites, pancreaticopleural fistula, and pseudocyst . The patient in our case has developed a pseudocyst with a well-defined wall. Management of a pseudocyst can include watchful waiting for asymptomatic lesions ; for symptomatic collections, percutaneous, surgical, or endoscopic strategies are available, and the choice should be made in a multidisciplinary fashion with the expertise of the particular institution in mind. Endoscopic drainage of a pseudocyst was first described by Gerald Rogers et al. in 1975 [32] with subsequent improvement by creating a fistula into the stomach by Richard Korazek in 1985 [33]. The methodology has since undergone several modifications.
Prior to starting a cystgastrostomy , consultation with a radiologist is often necessary, and at the minimum, careful review of abdominal CT and/or MRI should be done to ensure there is a mature wall around the fluid collection (Fig. 12.1b) and direct apposition of the stomach wall and the pseudocyst [34]. Of note, the radiology imaging should be a recent study obtained within 1–2 weeks of the planned procedure. A mature wall often develops in 4–6 weeks, although we have seen mature walls as early as 3 weeks [2]. An appropriate wall for a cystgastrostomy (and necrosectomy) is considered to be > 3 mm. While some centers consider the upper limit of wall thickness to be 1 cm since thicker walls are thought to increase the risk of complications [34], we do not limit our interventions to capsules less than 1 cm and have had technical success with much thicker walls.
Patients are given antibiotics prior to the procedure (usually intravenous ciprofloxacin and metronidazole). We use general anesthesia rather than conscious sedation to reduce the risk of aspiration when draining large amounts of fluid into the stomach and to allow maximal sedation if complications arise that require a longer duration of the procedure. We use CO2 insufflation as it is rapidly absorbed by the body at the end of the procedure. The initial step is identification of the location of the pseudocyst and finding an appropriate puncture site. While traditionally direct endoscopic visualization of a bulge into the gastric or duodenal lumen was used as a marker, currently most centers use EUS guidance [35–39]. EUS is important for better localization of the fluid collections when there is no large extra-luminal compression, identification, and avoidance of blood vessels at the access site, and confirmation of the lack of necrotic tissue. The best data supporting the use of EUS was a randomized controlled trial by Varadarajulu et al. [35] comparing EGD to EUS-guided cystgastrostomy of pseudocysts, where the use of ultrasound was associated with greater success (100 % versus 33 %, P < 0.001) and trend toward decreased complications. EGD-related complications included one death from massive hemorrhage of gastric varices at the puncture site not visualized during the EGD.
Once a site has been identified using a therapeutic linear echoendoscope, we use a 19-gauge needle to puncture into the pseudocyst (Fig. 12.6). We try to puncture at an angle that ideally allows the endoscope to be kept straight in the body of the patient, allowing forces to be transmitted directly to the wall of the pseudocyst. Fluid can be aspirated for bacteriological and culture studies. In addition, contrast is instilled into the pseudocyst to identify borders of the pseudocyst and to maintain distention. We over-inject contrast to expand the pseudocyst by 5–10 mm to keep the pseudocyst distended and obviate the need for electrocautery when trying to traverse the wall of the pseudocyst (In press data). A 0.035-inch guidewire is then coiled numerous times into the cavity, and the access site is dilated using hydrostatic balloons. We start with a 6-mm biliary-dilating balloon (typically the Hurricane Biliary Balloon Dilation Catheter, Boston Scientific, Marlborough, MA) and serially dilate to 15 mm. Alternative strategies for tract creation include brief use of cautery with a needle-knife sphincterotome or cystotome. These strategies, however, likely pose increased risk of bleeding due to inadvertent cutting of a gastric vessel. Double pigtail stents are then placed through the tract to allow adequate drainage. We generally use three 10Fr double pigtails for drainage, with length depending on the depth necessary to adequately drain the pseudocyst.
Fig. 12.6
Cystgastrostomy of patient in Case 1. a An appropriate path was found using EUS, devoid of any intervening vasculature. In addition, the purely liquid nature of the collection was confirmed. b A long guidewire was then placed in the collection and c the tract was dilated with a hydrostatic balloon. d One 10Fr × 7 cm and one 10Fr × 4 cm pigtail stents were placed into the pseudocyst to allow drainage
On follow-up (Fig. 12.7), we reimage our patients in 4–6 weeks with a CT scan or MRI to evaluate for continued contraction or resolution of the pseudocyst and the presence of the stents. If the pseudocyst has resolved and the stents remain in place, we perform an EGD to remove the stents, although a small randomized controlled study has shown that leaving stents in place even after resolution of pseudocyst may decrease recurrence of the collection [40]. In this small study, patients either had their stents removed at a median of 2 months following cystgastrostomy or 12 months later with 38 % (5/13 patients) recurring in the former group compared to no recurrences with long-term stent patients. If the stents have fallen out by themselves, no further procedure is needed. If the pseudocyst is resolving, more time is allowed for drainage and involution. If the stents have fallen out and the patient remains symptomatic with an ongoing pseudocyst, a further cystgastrostomy may be needed to adequately drain the pseudocyst. Finally, if there is no change or increase in size of the pseudocyst, we reevaluate for pancreatic duct disruption and possible stent blockage. Surgical referral may also be appropriate as discussed below.
Fig. 12.7
Sample algorithm for follow-up after cystgastrostomy of a pseudocyst or endoscopic necrosectomy of a walled-off pancreatic necrotic collection. Actual protocol should be tailored to the clinical presentation of each patient
Outcomes, Complications, and Alternative Treatment for Failures
When performed in the setting of an experienced multidisciplinary team, the initial success rate in creation of the cystgastrostomy is high, up to 94–95 %, with a pseudocyst resolution rate of 90–100 % [41–43]. Complications can occur in 0–20 % of patients and include bleeding sometimes requiring surgery usually due to the use of electrocautery without EUS guidance to enter the pseudocyst , inadequate drainage, and pseudocyst infection. There is minimal mortality (< 1 %) with at least one death related to electrocautery used to access the pseudocyst without EUS. Recurrences can range from 0 to 16 %, and at least one recent paper shows resolution of the pseudocyst in all patients and no recurrence over at least 24-month follow-up [41]. Of note, in this paper, patients undergoing endoscopic cystgastrostomy had stents routinely removed 2 months following the procedure if imaging demonstrated resolution of the pseudocyst. Success is significantly lower if there is necrotic debris in the collection, underscoring the importance of preprocedural evaluation with imaging and EUS and ensuring the collection is a pseudocyst. Hookey et al. [28] showed that success of cystgastrostomy with or without transpapillary stenting of a pseudocyst was over 88 %, compared to only 25 % if necrotic debris is present.