Table 1.
Case | Age (yr/d) | Sex | Country | Year | Underlying disease | TPN | MRI finding | Thiamin level | Thiamin treatment | Outcome |
---|---|---|---|---|---|---|---|---|---|---|
Bian et al. [11] | 75 yr | F | China | 2016 | SBS secondary to gastrectomy | PN dependent without thiamin | High T2 intensity in both mammillary bodies and bilateral thalamus | Undetectable | NA | Improvement within 10 d and complete resolution in 29 d |
Santarpia et al. [10] | 20 yr | F | Italy | 2009 | SBS secondary to gastrectomy | TPN dependent without thiamin | Bilateral and symmetrical hyper-intensities in the subthalamus | Undetectable | 100 mg IV daily | Improvement within 15 d and complete resolution in 40 d |
Benidir et al. [8] | 8 yr | F | Canada | 2014 | SBS secondary to gastroschisis | TPN dependent without thiamin supplementation for 3 months | Symmetric lesions of the basal ganglia, mammillary bodies and peri-aqueductal region of the midbrain | Undetectable | 100 mg IV daily | Complete resolution within 48 h |
Roilides et al. [9] | 3 yr | M | Greece | 2019 | SBS secondary to NEC gastrectomy for double volvulus | PN dependent with 3.1 mg thiamin | Bilateral symmetric hyper-intense signals in the peri-aqueductal area and both medial thalami | 10 μg/L (normal levels, 28–85) | 25 mg IM daily | Improvement within 48 hr and complete resolution in 96 hr |
Present case | 3 d | M | China | 2019 | SBS secondary to gastroschisis | PN dependent with 3.0 mg thiamin | Symmetric lesions of the basal ganglia, caudate nucleus head, thalamus medial margin symmetry | 19.7 nmol/L (normal levels, 41.5–108.9 nmol/L) | 100 mg IV daily | Improvement within 72 hr and complete resolution in 96 hr |
F – Female; IM – intramuscular; IV – intravenous; M – Male; MRI – magnetic resonance imaging; NA – not applicable; NEC – necrotizing enterocolitis; PN – parenteral nutrition; SBS – short bowel syndrome; TPN – total parenteral nutrition.