In the last few decades, an increase in acute pancreatitis (AP) cases in children and adults has been reported.1 AP affects 1/10,000 children annually, 30% of whom will develop recurrent acute pancreatitis (RAP) or chronic pancreatitis (CP).2 In Mexico, RAP has been estimated at 24-37% in children.3

The majority of guidelines are extrapolated from the adult population, which poses challenges, given that pediatric AP etiology differs substantially from that in adults.1,4

According to the Consensus on the Diagnosis and Treatment of Acute Pancreatitis in Children and Adolescents from the Asociación Mexicana de Gastroenterología, etiology varies according to age group and comorbidities and risk factors should be carefully assessed in the clinical approach.3 In the pediatric population, more than one contributing factor is often identified in the development of AP, RAP, and CP. Reported etiologies include genetic, anatomic, obstructive, pharmacotoxic, traumatic, metabolic, systemic, infectious, autoimmune, environmental, and idiopathic causes.2,3,5,6 Of the anatomic causes, pathogenic variants and malformations (pancreas divisum 14%, pancreatic loop 0.5%, annular pancreas < 1%) predispose to recurrent episodes.2 Other anatomical variations may occur at the level of the bile ducts, such as the cholecystohepatic duct, segmental variations of the bile duct, aberrant bile ducts, or ducts of Luschka (subvesicular or supravesicular ducts). Luschka ducts are small ducts originating from the biliary system, typically draining into the right hepatic lobe. There are theories on a congenital etiology, as well as an acquired origin, but there is little information on their pathophysiology, incidence, and prevalence, or their association with AP and RAP. In their study, Handra-Luca et al. reported on 55 cases of adult patients with accessory Luschka ducts, 11 of whom presented with pancreatitis (20%, 6 women, 5 men, 35-86 years of age).7

Given the scarcity of literature available on said association in the pediatric population, we consider the present case relevant, emphasizing the importance of a systematic clinical approach.

A previously healthy 11-year and 7 month-old girl, with no family history of autoimmune disease, eutrophic (weight 35.7 kg, height 149.5 cm, BMI 16 kg/m2, p17.8%, z-score –0.92), presented with a 3-month progression of sharp, non-radiating epigastric pain, accompanied by nausea, but no vomiting, and elevated pancreatic enzymes, consistent with 2 episodes of AP, separated by 3 months. She received symptomatic treatment. The initial ultrasound, computed tomography, and magnetic resonance imaging studies were unremarkable. The patient was referred to our center for etiologic evaluation (Table 1). She experienced 3 additional episodes of pancreatitis (at one-month intervals), classified as RAP due to clinical and biochemical resolution between episodes. Because of persistent recurrent episodes and the unavailability of genetic testing, endoscopic ultrasound-guided pancreatic biopsy was performed (Table 1). One month later, the patient had a sixth episode of pancreatitis, and for the first time, elevated transaminases (ALT 94.9 U/L, AST 49 U/L). An ultrasound study identified biliary sludge. With suspected biliary etiology, pediatric surgery was consulted to perform cholecystectomy. Intraoperative findings were a short cystic duct crossed by a duct of Luschka. Laparoscopic cholecystectomy was performed. The patient had no new episodes of pancreatitis during the 12-month follow-up period.

In cases of pancreatitis in children, especially cases classified as recurrent or chronic, a systematic and individualized diagnostic approach is essential. It favors timely detection and treatment, through ordering laboratory and imaging studies that enable etiology and severity to be identified and disease progression to be monitored (Table 2).1,8 In accordance with the proposals of the INSPPIRE study,1 our patient met the criteria for RAP: 2 distinct episodes of AP with an interval of clinical and biochemical resolution between the pancreatitis episodes. After a comprehensive clinical history, initial studies were ordered to rule out traumatic, metabolic, pharmacotoxic, and infectious causes. Imaging studies are useful for classification and for identifying the differential diagnoses, risk factors, and anatomic or obstructive abnormalities.1,4,8 To minimize radiation exposure, abdominal ultrasound is recommended as the first-line imaging study in children, for ruling out obstruction or anatomic alterations.8 The most useful finding is pancreatic duct dilatation (absent in our patient), which, depending on the findings, can be further evaluated through endoscopic ultrasound.

According to the current literature, if a biliary cause is identified (gallstones, microlithiasis, or biliary sludge) in the pediatric population, cholecystectomy is indicated during the same hospitalization, to prevent the recurrence of pancreatitis.5 In the present case, the postoperative outcome was favorable.

This report highlights the importance of a systematic diagnostic approach, and even when the INSPPIRE diagnostic criteria are taken into account,1 episodes of uncommon etiologies may occur that hinder providing a timely diagnosis and treatment, having an impact on recurrence and/or complications.