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Description

This case illustrates the multifactorial nature of medically complex pediatric patients with Duchenne Muscular Dystrophy (DMD) and their risk of malnutrition, leading to rare complications like superior mesentery artery syndrome (SMAS). This case is particularly notable due to significant barriers to accessing healthcare, including inability to obtain sufficient enteric feeds, inconsistent transportation, and incarceration of the primary guardian one month following SMAS resolution. Malnutrition in pediatric patients is complex, particularly when potential neglect is involved; however, this family also faced barriers to care, and it is unclear if the initial weight loss leading to SMAS was directly related to intentional neglect. Furthermore, this case highlights the impacts changes in guardianship may have on medically complex children, suggesting a need for better coordinated care.

A 14-year-old male with a known history of DMD, steroid dependence, developmental delay, and severe protein-calorie malnutrition presented to the emergency department for evaluation of worsening bilious emesis, abdominal pain, and persistent constipation over the previous 24 hours. He was hemodynamically stable, but weighed 22.1 kg after significant weight loss. Physical exam was notable for abdominal tenderness without distention. The patient was started on IV fluid bolus and IV ondansetron for symptom control.    Labs were notable for leukocytosis (WBC 12.1 x10⁹/L) with neutrophilic predominance, hypokalemia (K⁺ 2.4 mmol/L), decreased bicarbonate (19 mmol/L), and an elevated anion gap (26). Liver enzymes were mildly elevated, though the lactate was within normal limits. Computed tomography (CT) of the abdomen and pelvis demonstrated significant gastric distention with radiographic features consistent with SMAS. Additional findings included compression of the left renal vein.

This case underscores the complex interplay of neuromuscular disease, nutritional challenges, and multidisciplinary coordination in managing medically fragile pediatric patients.  This patient's experience illustrates the profound impacts social determinants of health have on disease trajectory, treatment adherence, and outcomes. Coordinated multidisciplinary care and proactive social support are critical to optimizing outcomes in similarly vulnerable populations, decreasing the risk of fractured medical care that may accompany placement disruption or loss of resources.

Publication Date

5-8-2026

Disciplines

Pediatrics

Comments

2026 Research Day Corewell Health West, Grand Rapids, MI, May 8, 2026. Abstract 2130

A Case of Superior Mesentery Artery Syndrome in a Patient with Duchenne Muscular Dystrophy

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