What is the next best step in the management?

Imaging-guided reduction
– avoids surgery
– absolute contraindications: perforation, peritonitis, hemodynamic instability
– pneumatic or hydrostatic – increases the intraluminal pressure in the colon
– under fluoroscopic or ultrasound guidance (US better because of the lack of ionizing radiation)

Hydrostatic reduction under ultrasound guidance was performed

Fluid-distended cecum with gaping ileocecal valve and reflux of fluid in the terminal ileum as a marker of successful reduction

Reference

Pušnik L, Slak P, Nikšić S, Winant AJ, Lee EY, Plut D. Ultrasound-guided hydrostatic reduction of intussusception: comparison of success rates between subspecialized pediatric radiologists and non-pediatric radiologists or radiology residents. Eur J Pediatr. 2023 Jul;182(7):3257-3264. doi: 10.1007/s00431-023-04987-1. Epub 2023 May 6. PMID: 37148276; PMCID: PMC10354123.

What is the most likely diagnosis?

Erdheim-Chester disease

Teaching points
– Erdheim-Chester disease (ECD) is a non-Langerhans cell histiocytosis characterized by multiorgan xanthomatous infiltration

– The diagnosis is based on clinical, imaging, and histopathological features

– Patients with ECD may present with bone pain, diabetes insipidus, exophthalmos, constitutional symptoms, interstitial lung disease, ureteral obstruction, renal impairment, cardiac dysfunction and tamponade, cerebellar or pyramidal symptoms, and xanthelasma.

– ECD has a wide range of manifestations throughout the body

– Skeletal involvement is the most common. At imaging, there is bilateral patchy or diffuse symmetric osteosclerosis of the lower extremity metaphyses and diaphyses, with relative sparing of the subchondral surfaces. Radiographically, cortical thickening, coarsened trabeculae, medullary sclerosis, and loss of the corticomedullary differentiation may be demonstrated

– Kidneys and retroperitoneum are often involved

– CT and MRI may show ‘hairy kidney sign’ that is demonstrated as irregular symmetric infiltration of the bilateral perirenal and posterior pararenal spaces

– Obstructive uropathy may result from medial displacement of the ureters

– Periaortic soft tissue is often shown, which is known as ‘coated aorta sign’

– Pulmonary involvement has been reported in 15-35% of patients with ECD and includes smooth interlobular septal thickening, micronodules, ground-glass opacities, thickening of interlobar fissures, and parenchymal consolidation

– Chest radiographs will often show interstitial edema pattern with cardiomegaly and pleural effusions that do not respond to diuretics 

– Orbital and central nervous system involvement are common findings. Retrobulbar masses that can cause proptosis and optic nerve edema may be present

– The hypothalamic-pituitary axis is the most common site affected within the central nervous system. Absence of the normal T1 hyperintense signal of the neurohypophysis occurs with enhancing nodular soft tissue of the pituitary stalk and posterior pituitary gland that results in central diabetes insipidus. Intra- and extra-axial cerebral and spinal lesions may be observed

– Treatment: Targeted therapy such as BRAF inhibitors, MEK inhibitors, interferon alfa, steroid therapy, radiotherapy, and surgery may be performed. There is no known cure for ECD and historically the prognosis has been poor 

– In our case, a biopsy from the perirenal soft tissue was performed. The histopathological findings confirmed the diagnosis of ECD

Describe the findings

*Marked biliary tree dilatation, common bile duct almost 2 cm wide with abrupt caliber change at the level of the pancreatic head
*No gallstones seen in biliary ducts or the distended gallbladder
*Diffuse pancreatic enlargement with marked restricted diffusion, no peripancreatic fat stranding, free fluid or collections
*Main pancreatic duct narrowing, barely visible

Differential diagnosis includes…

Differential diagnosis includes…

*Pancreatic cancer (especially diffuse infiltrative)
*Pancreatic lymphoma
*Autoimmune pancreatitis

Pancreatic biopsy confirmed autoimmune pancreatitis

Autoimmune pancreatitis is:
– Rare type of chronic pancreatitis
– Associated with IgG4-related sclerosing disease and autoimmune diseases
– Bulky appearance of the pancreas on imaging (“sausage shaped”), main pancreatic duct narrowing and absence of peripancreatic inflammatory changes seen in the acute pancreatitis
– Stenosis of the common bile duct is typical

What is the best course in treatment?

What is the best course in treatment?

Corticosteroids

Follow-up MRCP three weeks later showed improvement after corticosteroid therapy
Cholecystectomy was also performed