S3292 Recurrent Pancreatitis in Pediatric-Onset Median Arcuate Ligament Syndrome: A Case Report

Introduction: Median arcuate ligament syndrome (MALS) is a rare vascular compression disorder that predominantly affects young adults. The association between MALS and recurrent pancreatitis represents an uncommon but clinically significant complication. We present a case of pediatric-onset MALS with chronic recurrent pancreatitis spanning 15 years. Case Description/Methods: A 20-year-old woman with MALS diagnosed at age 5 presented with 3-4 days of intermittent diffuse abdominal pain and nausea. She reported annual episodes of pancreatitis since childhood. Physical examination revealed classic epigastric tenderness. Laboratory studies showed elevated lipase (279 U/L), leukocytosis (12.1 × 10³/μL), and normal lipid panel. Computed tomography abdomen/pelvis demonstrated no acute findings. Her extensive surgical history included 2 failed celiac artery stent placements with subsequent removal due to complications, and unsuccessful arcuate ligament decompression surgery. Multiple comprehensive pancreatitis workups remained negative for alternative etiologies. The patient was managed conservatively with intravenous fluid resuscitation, diet advancement, and symptomatic treatment. She achieved clinical improvement and was discharged with gastroenterology follow-up. Discussion: This case illustrates the challenging management of MALS-associated recurrent pancreatitis. The patient's 15-year history of annual pancreatitis episodes beginning in early childhood represents an unusual presentation of MALS complications. Despite multiple interventional attempts including endovascular and surgical approaches, her symptoms persisted, highlighting the refractory nature of some MALS cases. The negative comprehensive pancreatitis evaluations support MALS as the primary etiology. Conservative management remains the mainstay when surgical interventions fail. This case emphasizes the need for multidisciplinary care and continued research into alternative therapeutic approaches for complex MALS presentations with associated pancreatic complications.

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Publication Details

Journal
The American Journal of Gastroenterology
Published
2025-10-01
DOI
https://doi.org/10.14309/01.ajg.0001140628.53874.46
Primary Topic
Endometriosis Research and Treatment
Type
article
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article

S3292 Recurrent Pancreatitis in Pediatric-Onset Median Arcuate Ligament Syndrome: A Case Report

B J Byun, Camila Villacreses, Sharifah Aishah Wan, Amanda Rigdon et al.
The American Journal of Gastroenterology
Endometriosis Research and Treatment
article

S3292 Recurrent Pancreatitis in Pediatric-Onset Median Arcuate Ligament Syndrome: A Case Report

B J Byun, Camila Villacreses, Sharifah Aishah Wan, Amanda Rigdon, Omar Zuhdi
article en

Abstract

Introduction: Median arcuate ligament syndrome (MALS) is a rare vascular compression disorder that predominantly affects young adults. The association between MALS and recurrent pancreatitis represents an uncommon but clinically significant complication. We present a case of pediatric-onset MALS with chronic recurrent pancreatitis spanning 15 years. Case Description/Methods: A 20-year-old woman with MALS diagnosed at age 5 presented with 3-4 days of intermittent diffuse abdominal pain and nausea. She reported annual episodes of pancreatitis since childhood. Physical examination revealed classic epigastric tenderness. Laboratory studies showed elevated lipase (279 U/L), leukocytosis (12.1 × 10³/μL), and normal lipid panel. Computed tomography abdomen/pelvis demonstrated no acute findings. Her extensive surgical history included 2 failed celiac artery stent placements with subsequent removal due to complications, and unsuccessful arcuate ligament decompression surgery. Multiple comprehensive pancreatitis workups remained negative for alternative etiologies. The patient was managed conservatively with intravenous fluid resuscitation, diet advancement, and symptomatic treatment. She achieved clinical improvement and was discharged with gastroenterology follow-up. Discussion: This case illustrates the challenging management of MALS-associated recurrent pancreatitis. The patient's 15-year history of annual pancreatitis episodes beginning in early childhood represents an unusual presentation of MALS complications. Despite multiple interventional attempts including endovascular and surgical approaches, her symptoms persisted, highlighting the refractory nature of some MALS cases. The negative comprehensive pancreatitis evaluations support MALS as the primary etiology. Conservative management remains the mainstay when surgical interventions fail. This case emphasizes the need for multidisciplinary care and continued research into alternative therapeutic approaches for complex MALS presentations with associated pancreatic complications.

The American Journal of GastroenterologyVol. 120(10S2)
Openalex Percentile: Top 60%
Endometriosis Research and Treatment
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