Pediatr Gastroenterol Hepatol Nutr.  2019 Nov;22(6):588-593. 10.5223/pghn.2019.22.6.588.

Endovascular Closure Resolves Trimethylaminuria Caused by Congenital Portosystemic Shunts

• Ponce-Dorrego MD
• Garzón-Moll G

• KMID: 2462100
• DOI: http://doi.org/10.5223/pghn.2019.22.6.588

Abstract

This study aimed to report three new cases of an association between two rare conditions, congenital portosystemic shunts (CPSS) and trimethylaminuria (TMAU), and the efficacy of endovascular closure of the CPSS for resolving TMAU. Between November 2014 and April 2017, 15 patients with CPSS were enrolled in this prospective study to assess the efficacy of percutaneous endovascular shunt closure. Three patients presented with clinical symptoms of TMAU that were confirmed by urine analysis of trimethylamine (TMA) and TMA n-oxide. One year after endovascular closure of the congenital portosystemic shunt, the same parameters were evaluated were obtained and the values were compared to the pretreatment values. The results indicated the disappearance of clinical symptoms of TMAU and normalization of the urine test parameters in two patients and no changes in one patient, who developed new portosystemic communications.

Keyword

Congenital portosystemic shunt; Trimethylaminuria; Interventional radiology

MeSH Terms

Humans
Portasystemic Shunt, Surgical*
Prospective Studies
Radiology, Interventional

Figure

• 

  Fig. 1 Urine levels of TMA (A) and TMAO (B) expressed as μmoL/mmoL creatinine before treatment (‘1’) and 1 year after treatment (‘2’). TMA: trimethylamine, TMAO: trimethylamine n-oxide.

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  Fig. 2 TMA/TMAO (A) and % of excretion of TMAO (B) before treatment (‘1’) and 1 year after treatment (‘2’). TMA: trimethylamine, TMAO: trimethylamine n-oxide.

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