Assessment of aortomesenteric distance and mesenteric and retroperitoneal adipose tissue thickness in genetic forms of lipodystrophy

Introduction Lipodystrophy is a rare disease characterized by the loss of adipose tissue. Visceral adipose tissue loss in certain forms of lipodystrophy may affect the amount of mesenteric fat.

Method We studied visceral adipose tissue by measuring the thickness of mesenteric and retroperitoneal adipose tissue and the aortomesenteric (AOM) distance in patients with genetic forms of lipodystrophy (n=48; 7 males; 41 females; mean age 39.111.9 years; 19 with congenital generalized lipodystrophy [CGL], and 29 with familial partial lipodystrophy [FPLD]). An age- and gender-matched control group with a ratio of 1:2 was generated.

Results Patients with CGL had severely depleted mesenteric adipose tissue (2.0 [IQR: 1.5-3.5] mm vs. 18.8 [IQR: 4.4-42.2] mm in FPLD, P<.001; 30.3 [IQR: 13.9-46.6] mm in controls, P<.001) and retroperitoneal adipose tissue (1.3 [IQR: 0.0-5.3] mm vs. 33.7 [IQR: 21.6-42.1] mm in FPLD, P<.001; 29.7 [IQR: 23.1-36.7] mm in controls, P<.001). The AOM distance was shorter in patients with CGL (8.1 [IQR: 6.0-10.8] mm) compared to patients with FPLD (vs. 13.0 [IQR: 8.8-18.1] mm; P=.023) and controls (vs. 11.3 [IQR: 8.4-15.5] mm, P=.016). Leptin levels were positively correlated with AOM distance in lipodystrophy (r=.513, P<.001). Multivariate linear regression analysis identified body mass index as a significant predictor of AOM distance (data controlled for age and sex; beta=0.537, 95% CI: 0.277-0.798, P<.001). Twelve of 19 patients (63%) with CGL had an AOM distance of <10 mm, a risk factor that may predispose patients to developing superior mesenteric artery syndrome.

Conclusion CGL is associated with a severe loss of mesenteric adipose tissue, which leads to a narrowing of the space between the superior mesenteric artery and the aorta.