Therapeutic endoscopic ultrasound (tEUS) has firmly cemented itself as a viable tool for several gastrointestinal disorders in adults. Despite a limited diffusion, partially related to a difficult learning process in a pediatric setting,¹ tEUS is now an emerging option also for children and it is relevantly altering the common surgical management of some nutritional issues and acute disorders in favor of less invasive management strategies. To explicit this emerging trend, three clinical cases recently managed in our multi-disciplinary team will be presented.

Background

Gastroparesis is the most common cause of pediatric gastric outlet obstructions (GOO) and is often associated with oropharyngeal dysphagia, bloating, retching, vomit and gastro-esophageal reflux disease (GERD) in children with neurodevelopmental disabilities (NPDC). A significant undernutrition is reported in 13% to 52% of NPDC, requiring a prompt gastric decompression together with a stable jejunal access for nutrition. Surgical gastrojejunostomy (SJ) with percutaneous endoscopic gastrostomy (PEG) could represent a possible long-term solution but is limited by a greater invasiveness, unfriendly anatomy and comorbidities. Percutaneous endoscopic gastrojejunostomy (PEGJ) is the preferred option for NPDC.² J-Tube clogging and retrograde dislodgement are very common in this kind of patients: the average duration of PEGJ in NPDC is 39 days.³

Multiple series have documented favorable outcomes in adults treated with endoscopic ultrasound-guided gastrojejunostomy (EUS-GJ) for the treatment of malignant and benign GOO as a non-operative alternative. Technical and clinical success are respectively 92% and 86%.⁴ A video case report about re-routing a PEGJ through an EUS-GJ to prevent retrograde J-tube dislodgments in NPDC was recently published by our group.⁵

Clinical case

A 17-year-old boy (28 kg of weight) with severe neurological impairment and a dystonic syndrome resulting from neonatal kernicterus under jejunal feeding was referred to our attention for repeated PEGJ tube dislodgments requiring repeated hospital admissions and prolonged parenteral nutrition. Severe scoliosis with osteopenia, delayed gastric emptying, retching and GERD were associated. SJ and direct percutaneous endoscopic jejunostomy were considered as secondary options.

Description of the procedure

Under general anesthesia, a linear echoendoscope (Pentax EG38-J10UT; PENTAX Europe GmbH, Hamburg, Germany) is employed. Procedure steps are described in Fig. 1–4.

Figure 1. To identify the correct target jejunal loop, a direct assisted method with saline mixed with contrast and indigo carmine injected by a diagnostic catheter was employed. To prevent retrograde filling solution from the target jejunal loop to the stomach, anti-peristaltic drugs and limited volumes are suggested.

Figure 2. A cautery-tipped, 15 mm × 10 mm lumen apposing metal stent (Hot Axios; Boston Scientific, Marlborough, MA, USA) was placed to create the gastrojejunostomy (GJ) under endoscopic ultrasound (EUS) and fluoroscopy guidance. As the EUS GJ will support percutaneous endoscopic gastrojejunostomy-tube re-routing, it is recommended to perform the gastric puncture not closer than 3 cm from the percutaneous endoscopic gastrostomy bumper.

Figure 3. Few months later, the lumen apposing metal stent (LAMS) was removed, and the gastrojejunostomy could be intubated with a standard gastroscope. To prevent obliteration of the endoscopic ultrasound-gastrojejunostomy by surrounding mucosa, a cautery ablation of the fistula tract after LAMS removal may be considered.

Figure 4. The percutaneous endoscopic gastrojejunostomy tube was placed from the stomach into the efferent jejunal loop through the endoscopic ultrasound-gastrojejunostomy. Contrast injection or alternative technique may be necessary to confirm that the isoperistaltic bowel loop is intubated.

Results

Three months after re-routing, a 55% of caloric intake tolerance by jejunal feeding and a 5 kg weight improvement with a progressive autonomization from parenteral nutrition were obtained. At one year follow up after the re-routing, no further dislodgments have been reported and the nutritional target is mainly reached with jejunal feeding.

Background

Aortomesenteric syndrome (AOMS) is a rare condition (incidence 0.1%–0.3%) determined by an obstruction of the third duodenum between the abdominal aorta and the superior mesenteric artery, promoted by a sudden reduction of the mesenteric fat pad between the two arteries.⁶ Any severe malnourishment, caused by hypermetabolic state, dietary problems (e.g., anorexia nervosa or motility disorders) or cachexia (e.g. AIDS, cancer, paraplegia) can be complicated by AOMS. Clinical presentation is characterized by abdominal pain, vomiting (with nocturnal worsening) and further weight loss.⁶

The diagnosis requires a high level of suspicion, based on clinical symptoms and radiologic evidence of obstruction. An aortomesenteric angle of ≤ 25° is strongly suggestive for AOMS syndrome. Retention of barium within the duodenum, characteristic vertical linear extrinsic pressure on the third portion of the duodenum, and duodenal dilation with gastric dilation are also supportive for the diagnosis. Abdominal X-ray, computed tomography (CT) scan and magnetic resonance imaging (MRI) can confirm the clinical suspect of AOMS but are limited by low sensibility and inadequate compliance in small children.⁷

Initial management is based on weight recovery strategies together with gastro-duodenal decompression and electrolytes balance.⁸ Laparoscopic duodenojejunostomy is reserved for cases refractory to conservative management.⁹ PEGJ with the jejunal extension above the compass may represent a minimally invasive approach to ensure gastroduodenal decompression together with enteral feeding.¹⁰ To facilitate the jejunal tube placement in patient with gastroduodenal dilatation, an endoscopic gastropexy can be placed to support a direct trans-gastric over-the-wire tube placement by using an ultrathin endoscope.

EUS has been already employed in AOMS.^11,12 It provides the most reliable and repeatable measure of the aortomesenteric angle which is useful both for diagnosis of syndrome and to monitor the progressive weight regain. This evaluation can be performed during the same procedural sedation necessary to support the enteral feeding access placement and management. EUS-GJ has also been reported as an alternative to surgery for gastric decompression in AOMS.^13–15 We will describe a successful EUS-based conservative management of one girl with AOMS, treated by direct PEGJ by-passing the vascular stricture.

Clinical case

A 14-year-old female with hyperthyroidism and a recent history of cyclic vomit, food refusal, relentless post-prandial abdominal pain and significant weight-loss (almost 12 kg in four months) was admitted to our department. A barium swallow demonstrated a severe gastroduodenal dilation. Upper endoscopy detected severe stasis esophagitis and gastroduodenitis. Two endoscopic attempts to place nasojejunal feeding tube failed because of the severe dilatation. An abdominal MR demonstrated a 4 mm aortomesenteric distance between and AOMS was suspected. An EUS-assisted PEGJ placement was offered to the family as an alternative to laparoscopic duodenojejunostomy.

Description of the procedure

Under general anesthesia, a slim linear echoendoscope (EG34-J10UT; PENTAX Europe GmbH) and an ultrathin gastroscope (XP190N; Olympus Europe, Hamburg, Germany) is employed. Procedure steps are described in Fig. 5–8.

Figure 5. An aortomesenteric angle of 19° and vascular compression to the 3rd duodenum were confirmed. An orotracheal prompt intubation is recommended to reduce the risk of aspiration in severe gastric outlet obstructions.

Figure 6. A safe-triangle was identified on the abdominal wall to create the gastropexy under endoscopic ultrasound guidance. Inflation of the stomach is necessary to create the gastropexy, thus, previous marking the safe triangle on the abdomen is recommended.

Figure 7. Gastropexy was created by placing 3 t-fasteners on the vertices of the safe-triangle. The gastro-cutaneous fistula was created and dilated over-the-wire with an introducer. Definite closure of the t-fastener anchoring suture can be performed at the end of the procedure to avoid excessive tension and buried syndrome.

Figure 8. An ultrathin endoscope was advanced from the gastro-cutaneous fistula into the 3rd duodenum and the percutaneous endoscopic gastrojejunostomy (PEGJ) tube was released over-the-wire. Contrast can be injected from the PEGJ tube to confirm that the enteral tube is placed beyond the vascular stricture.

Results

Three months after jejunal and oral feeding, together with gastric decompression EUS was repeated and an aortomesenteric angle of 44° was demonstrated. As a gastric scintigraphy confirmed a normal gastric emptying, during the same procedural sedation, the PEGJ was replaced by a low-profile PEG, which will be removed in the next few months.

Pancreatic fistula and chronic pancreatitis are common indications for endoscopic retrograde cholangiopancreatography (ERCP) in tertiary referral pediatric centers.¹⁶

Pancreatitis may be complicated by pancreatic fluid collections (PFC), walled-off pancreatic necrosis and disconnected pancreatic duct syndrome.¹⁷ Indication for an early PFC drainage may be represented by gastric outlet obstruction, biliary obstruction, infection, rupture, thrombosis and pseudoaneurysm.¹⁸

An adequate nutritional support, together with a negative pressure to the fluid collection ensured by activated peristalsis can be obtained with an early nose-jejunal feeding when gastric feeding is not tolerated.¹⁹

Clinical case

A 10-year, boy with 45 kg of weight and a clinical history of recurrent abdominal pain and constipation was admitted with coercive vomit, abdominal pain and elevate amylase and lipase. Abdominal ultrasound confirmed an acute pancreatitis with multiple and diffuse abdominal fluid collections.

A conservative management based on antibiotics, hyperhydration, pain killers and nasogastric decompression was attempted without a relevant clinical improvement. Five days after admittance, a CT scan demonstrated a 10 cm diameter PFC determining a GOO and extending from the pancreatic tail and splenic hilum to the mesocolon and surrounding the gastric greater curvature. As main pancreatic duct was dilatated and tortuous and a reduction of the pancreatic gland’s profile were also noted, a chronic pancreatitis complicated by a pancreatic duct rupture was suspected. Genetic pancreatitis and cystic fibrosis were investigated.

We decided for an early combined approach with ERCP, EUS drainage and jejunal tube.

Description of the procedure

Under general anesthesia, a linear echoendoscope, a standard duodenoscope and an ultrathin gastroscope are employed. Procedure steps are described in Fig. 9–14.

Figure 9. As the etiology of pancreatitis was undetermined, a double wire-assisted sphincterotomy was performed. Cholangiogram was negative. Selective cannulation excluded a pancreatobiliary mal-junction. As the access to diagnostic cholangio magnetic resonance is limited in children, a careful evaluation of the biliopancreatic junction is recommended before the sphincterotomy.

Figure 10. Wirsungography demonstrated a leakage from a secondary branch of the pancreatic tail and a marked chronic pancreatitis (Cambridge endoscopic retrograde cholangio-pancreatography based classification). After a low-pressure contrast injection to evaluate the pancreatic duct anatomy, an over-the-wire stone-extraction balloon can be employed to obliterate the duct and confirm the leakage.

Figure 11. A pancreatic stent (5-Fr, 5 cm) was placed into the pancreatic head to protect the pancreatic sphincterotomy and to reduce pancreatic juice flow. If duct’s anatomy doesn’t support the placement of a long stent to exclude the injured secondary branch, similar results can be obtained with short stent.

Figure 12. During the same procedural sedation, the scope was changed to linear endoscopic ultrasound and the evaluation from the stomach confirmed a 10 cm diameter, bilobated pancreatic fluid collections with debris. Doppler can be employed to select a safe tract for drainage avoiding blood vessels injuries.

Figure 13. Under endoscopic ultrasound and X-ray guidance, a trans-gastric drainage with two double pigtails (10-Fr, 5 cm) was established. An immediate outflow from the pancreatic fluid collections into the stomach was appreciated. Once the first double pigtail is placed with cystotome, an over-the-wire sphincterotome can be employed to enlarge the gastric entry for the second pigtail which will stabilize the cysto-gastrostomy.

Figure 14. At the end of the combined procedure, a naso-jejunal tube was left to establish a fast-track re-feeding and to stimulate the bowel peristalsis. Trans-nasal gastroscope can be employed to support an easier.

Results

A CT scan performed 48 hours after the procedure (Fig. 14), confirmed the correct placement of pancreatic stent, jejunal tube and pigtails. A reduction of 75% of the PFC’s volume was demonstrated and clinical improvement was also confirmed by labs and pain relief. The clinical course was complicated by a transient liver impairment, requiring vitamin k and plasma infusion. These findings were supportive for a moderate pancreatic exocrine insufficiency confirmed by low fecal elastase and treated by oral enzyme substitution.

The boy tolerated a progressive oral refeeding and was discharged 27 days later, after the removal of the two double-pigtails. An ERCP to remove the plastic stent and evaluate the pancreatic duct is scheduled after 3 months.

Several established indications of therapeutic endoscopic ultrasound can be successfully adjusted to the field of pediatrics to support a more effective and personalized management in relatively common disorders such as GOO or complicated pancreatitis.

A continuous cooperation with high volume centers is advocated to ensure a smooth adaptative process.

None.

No potential conflict of interest relevant to this article was reported.