Surgical indications for patients with hyperammonemia

Role of per-rectal portal scintigraphy in long-term follow-up of congenital portosystemic shunt

“…Surgical repair or embolization may be recommended for extrahepatic CPSS without closure because of the high risk of hepatopulmonary syndrome, pulmonary hypertension, and hepatic encephalopathy (4,7,8,(13)(14)(15)(16). Stringer (8) described that CPSS-affected individuals are at risk of developing hepatic encephalopathy and/or an intrahepatic tumor depending largely on the volume and duration of the shunt.…”

“…CPSS is generally suspected if serum total bile acid (TBA) and ammonia levels are elevated, and it is diagnosed by using color Doppler ultrasonography, dynamic contrast-enhanced computed tomography (CT), and per-rectal portal scintigraphy (PRPS) (7,(9)(10)(11)(12). Some shunts close spontaneously, whereas others need to be closed surgically or with embolization because of hyperammonemia leading to severe hepatic encephalopathy (7,8,(13)(14)(15). Therefore, it is important to follow up CPSS patients carefully with color Doppler ultrasonography, dynamic contrast-enhanced CT, and blood tests.…”

Successful preemptive surgical division of type 2-congenital extrahepatic portosystemic shunt in children

“…Uchino, et al identified 51 cases of congenital portosystemic shunt in Japan ; 34 of the intrahepatic type and 17 of the extrahepatic type, of which spontaneous closure was occurred only in the intrahepatic type, but not in the extrahepatic type (17). If patients with CEPS are left untreated, they will sustain various consequences such as repeated encephalopathy (7,18), neurocognitive dysfunction (14), pulmonary hypertension (19), hepatopulmonary syndrome (9), cataract (3), liver atrophy (16), liver tumors (10,14) and so on. Eroglu, et al reported damage incurred during development in childhood could reduce the functional capacity of the adult (14).…”

“…Eroglu, et al reported damage incurred during development in childhood could reduce the functional capacity of the adult (14). These shunts should be preemptively occluded by either surgical or radiological intervention after ruling out severe associated anomalies such as cardiac defects or maldevelopment of the intrahepatic portal as well as hepatic veins (4,6,7).…”

“…1A. Shunt ratios were determined by portal scintigraphy using 123 Iiodoamphetamine per rectum (7). The ratios were calculated as follows : counts obtained in the lung divided by total counts obtained in the liver and lung.…”