Abstract
Congenital intrahepatic portosystemic shunts are rare vascular malformations in which abnormal communications are created between the portal veins and the hepatic veins or the inferior vena cava system. Diagnosis is made by prenatal or postpartum ultrasound. Published data regarding presentation, symptoms, and prognosis is scarce. This study aimed to better understand the natural history and the course of the intrahepatic portosystemic shunts. Data were collected from children in two medical centers who were diagnosed with congenital intrahepatic portosystemic shunts on either prenatal or postnatal sonographic screening. The subjects’ medical information was collected including demographics, medical background, and sonographic and clinical outcome. Blood test results including ammonia levels and liver function tests were documented, as well as the sonographic dimensions of the shunt vessels and the spleen size. The data were analyzed using various statistical methods. Twenty-three children with portosystemic shunts were found and reviewed. Eight children were excluded from the study since records and follow-up were insufficient. Fifteen patients were included in the study (six females). All had intrahepatic shunt diagnosed either by prenatal screening or postnatal abdominal ultrasound and had more than one ultrasound and repeated blood tests. Shunt closure was observed in all children within a mean of 114.31 ± 115.05 days (median 84). There was no correlation between liver enzymes, ammonia, and ultrasound vascular and splenic diameters to time to closure. None of the children had any hepatic or other sequelae.
Conclusions: Our study suggests that congenital intrahepatic portosystemic shunt is a benign, self-limiting condition in which no correlation between the size of the shunt and the blood ammonia level to the outcome of the shunt was found. This is the first study that correlated radiological measures to the outcome. These results suggest that the treating physician should reassure families and conduct minimal follow-up and interventions in children with such conditions. Further, larger and prospective studies should be done to corroborate these conclusions.
What is Known: • Characteristics and natural history of intrahepatic portosystemic shunts are less defined. • The natural course of the intrahepatic malformations varies, but spontaneous, self-resolution of small shunts, usually occures within 1 to 2 years. | |
What is New: • In this study, congenital intrahepatic portosystemic shunt was shown to be benign, self-limiting condition in which all shunts closed within 3 months. • No correlation between the size of the shunt and the blood ammonia level to the outcome of the shunt was found. |
Similar content being viewed by others
Data availability
The data is available upon request.
Abbreviations
- LPV-LHV:
-
Left portal vein to left hepatic vein
- LPV-MHV:
-
Left portal vein to middle hepatic vein
- LPV-RHV:
-
Left portal vein to right hepatic vein
- RHV:
-
Right hepatic vein
- RPV-RHV:
-
Right portal vein to right hepatic vein
- SGA:
-
Small for gestational age
- US:
-
Ultrasound
References
Stringer MD (2008) The clinical anatomy of congenital portosystemic venous shunts. Clin Anat 21(2):147–157
Bernard O, Franchi-Abella S, Branchereau S, Pariente D, Gauthier F, Jacquemin E (2012) Congenital portosystemic shunts in children: recognition, evaluation, and management. Semin Liver Dis 32(4):273–287
Morgan G (1994) Superina R Congenital absence of the portal vein: two cases and a proposed classification system for portasystemic vascular anomalies. J Pediatr Surg 29(9):1239–1241
Baiges A, Turon F, Simon-Talero M et al (2020) Congenital extrahepatic portosystemic shunts (Abernethy malformation): an international observational study. Hepatology 71(2):658–669
McLin VA, Franchi Abella S, Debray D, Guérin F, Beghetti M, Savale L, Wildhaber BE, Gonzales E, Members of the International Registry of Congenital Porto-Systemic Shunts (2019) Congenital portosystemic shunts: current diagnosis and management. J Pediatr Gastroenterol Nutr 68(5):615–622
Kim MJ, Ko JS, Seo JK, Yang HR, Chang JY, Kim GB, Cheon JE, Kim WS (2012) Clinical features of congenital portosystemic shunt in children. Eur J Pediatr 171(2):395–400
Paganelli M, Lipsich JE, Sciveres M, Alvarez F (2015) Predisposing factors for spontaneous closure of congenital portosystemic shunts. J Pediatr 167(4):931–935 e12
Delle Chiaie L, Neuberger P, Von Kalle T (2008) Congenital intrahepatic portosystemic shunt: prenatal diagnosis and possible influence on fetal growth. Ultrasound Obstet Gynecol 32(2):233–235
Francois B, Gottrand F, Lachaux A, Boyer C, Benoit B, de Smet S (2017) Outcome of intrahepatic portosystemic shunt diagnosed prenatally. Eur J Pediatr 176(12):1613–1618
Ferrero GB, Porta F, Biamino E, Mussa A, Garelli E, Chiappe F, Veltri A, Silengo MC, Gennari F (2010) Remittent hyperammonemia in congenital portosystemic shunt. Eur J Pediatr 169(3):369–372
Gallego C, Miralles M, Marin C et al (2004) Congenital hepatic shunts. Radiographics 24(3):755–772
Han BH, Park SB, Song MJ, Lee KS, Lee YH, Ko SY, Lee YK (2013) Congenital portosystemic shunts: prenatal manifestations with postnatal confirmation and follow-up. J Ultrasound Med 32(1):45–52
Rosenberg HK, Markowitz RI, Kolberg H, Park C, Hubbard A, Bellah RD (1991) Normal splenic size in infants and children: sonographic measurements. AJR Am J Roentgenol 157(1):119–121
Sokollik C, Bandsma RH, Gana JC et al (2013) Congenital portosystemic shunt: characterization of a multisystem disease. J Pediatr Gastroenterol Nutr 56(6):675–681
Clyman RI, Mauray F, Roman C et al (1980) Circulating prostaglandin E2 concentrations and patent ductus arteriosus in fetal and neonatal lambs. J Pediatr 97(3):455–461
Author information
Authors and Affiliations
Contributions
RC—performed the US, writing and editing of the manuscript; MS—patient’s care, writing and editing of the manuscript; KK—data collection; ES—study intiation and design, patient’s care and wrote the manuscript.
Corresponding author
Ethics declarations
Ethics approval and consent to participate
This study was approved by the local IRB committee of Shaare Zedek Medical Center and Haddasha Medical Center. The IRB committee provided a waiver to consent the participants as this study was retrospective.
Consent for publication
N/A
Conflict of interest
The authors declare no conflict of interest.
Code availability
N/A
Additional information
Communicated by Peter de Winter
Publisher’s note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
About this article
Cite this article
Cytter-Kuint, R., Slae, M., Kvyat, K. et al. Characterization and natural history of congenital intrahepatic portosystemic shunts. Eur J Pediatr 180, 1733–1737 (2021). https://doi.org/10.1007/s00431-021-03949-9
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00431-021-03949-9