Two hyperandrogenic adolescent girls with congenital portosystemic shunt

Abstract

We describe two adolescent girls with a congenital portosystemic shunt who exhibited hyperandrogenism in addition to insulin resistant hyperinsulinaemia. Case 1 was referred to our clinic to undergo a routine clinical work-up prior to tonsillectomy at 14 years of age. Mild liver dysfunction was identi®ed and hypogenesis of the portal vein with a congenital portosystemic shunt diagnosed. Primary amenorrhoea and virilization were evident and an endocrinological evaluation revealed hyperandrogenism and insulin resistant hyperinsulinaemia. Case 2 was referred at 15 years of age because of cardiomegaly. Mild liver dysfunction and hyperbilirubinaemia led to a diagnosis of agenesis of the portal vein with a congenital portosystemic shunt. Virilization was evident and an endocrinological evaluation revealed hyperandrogenism and insulin resistant hyperinsulinaemia. The haemodynamics of these patients were similar to those of secondary portosystemic shunt due to liver cirrhosis, which is often associated with hyperinsulinaemia and/or non-insulin dependent diabetes mellitus. On the other hand, hyperandrogenism is associated with certain insulin-resistant conditions with hyperinsulinaemia, including the polycystic ovary syndrome (PCO). Hyperinsulinaemia is believed to cause hyperandrogenism in patients with PCO by stimulating androgen production in both the ovary and adrenal gland. Therefore, in congenital portosystemic shunts, hyperinsulinaemia is also thought to cause hyperandrogenism due to the same mechanism. Conclusion A certain percentage of female patients with hyperandrogenism, likely including those with polycystic ovary syndrome may also have congenital portosystemic shunts. Our results indicate that serum levels of total bile acids and ammonia are prognostic indicators of this hepatic vascular anomaly. Key words Hyperandrogenism á Hyperinsulinaemia á Portosystemic shuntAbbreviations D 4 A D 4 androstenedione á DHEA dehydroepiandrosterone á OGTT oral glucose tolerance test á PCO polycystic ovary syndrome á TBA total bile acids Eur J Pediatr (2001) 160: 307±311 Ó Springer-Verlag 2001

Asymptomatic Aneurysmal Portosystemic Venous Shunt: A Case Report and Review of the Literature

“…Some authors postulates a persistent venous anastomosis such as ductus venosus and right vitelline vein. Others advocate an acquired cause from rupture of a portal venous aneurysm into the hepatic vein or from a dilated heptic vein communicating with the inferior vena cava via inferior phrenic and suprarenal vein (5,10,14,19,21,28,29). Persistent ductus venosus could be a remote possibility in our case.…”

Insight into congenital absence of the portal vein: Is it rare?

“…Most frequently encountered Cardiovascular lesions, most frequently encountered in CAPV patients with Goldenhar syndrome, are tetralogy of Fallot and VSD [46] . Cardiomegaly has been noted in CAPV patients [41,47] , which may result from congenital insult or increased blood flow into the heart with a certain degree of congestive heart failure. A large number of non-heart abnormalities are found in all the cases reviewed [ malformations have been reported, such as double SV [1] , double inferior vena cava [40] , left sided IVC [2,17,26] , hepatic artery originating from superior mesenteric artery [8,50] , intrapulmonary shunting [7] , azygos and hemiazygos continuation [1,17,43,48] , and skin hemangioma [14,43] .…”

“…Although most cases do not possess any liver abnormalities [1,3,9,27,[43][44][45][47][48][49]54,57] , CAPV patients suffer from different levels of LD [2][3][4]9,15,16,20,23,24,[26][27][28][29]34,37,38,44,47,55,56] possibly due to the lack of portal flow. Hepatic encephalopathy, hepatopulmonary syndrome (HPS), and hepatorenal syndrome are closely related to metabolic disorder because of liver lesions, including hyperammonemia [3,27,41,59] and g alactosemia [20,27] .…”

“…CAPV patients also could have chronic renal failure [33,34] . Gonadal hormonal disorder [33,47,66] can result in hypergalactocemia [66][67][68] , primary amenorrhoea and signs of virilization [33] . Satoh's patients present with hyperandrogenism, insulin resistant hyperinsulinaemia, and hyperglycaemia [47] .…”

“…Gonadal hormonal disorder [33,47,66] can result in hypergalactocemia [66][67][68] , primary amenorrhoea and signs of virilization [33] . Satoh's patients present with hyperandrogenism, insulin resistant hyperinsulinaemia, and hyperglycaemia [47] . A few cases of PH [9,16,55] , rectal bleeding [9,21,33,42] , anaemia [31,33] and peripheral edema [21] have also been reported.…”