Management and classification of type II congenital portosystemic shunts

doi:10.1016/j.jpedsurg.2010.11.009

Journal of Pediatric Surgery

Volume 46, Issue 2, February 2011, Pages 308-314

https://doi.org/10.1016/j.jpedsurg.2010.11.009 Get rights and content

Abstract

Background

Congenital portosystemic shunts (PSS) with preserved intrahepatic portal flow (type II) present with a range of clinical signs. The indications for and benefits of repair of PSS remain incompletely understood. A more comprehensive classification may also benefit comparative analyses from different institutions.

Methods

All children treated at our institution for type II congenital PSS from 1999 through 2009 were reviewed for presentation, treatment, and outcome.

Results

Ten children (7 boys) with type II PSS were identified at a median age of 5.5 years. Hyperammonemia with varying degrees of neurocognitive dysfunction occurred in 80%. The shunt arose from a branch of the portal vein (type IIa; n = 2), from the main portal vein (type IIb; n = 7), or from a splenic or mesenteric vein (type IIc; n = 1). Management included operative ligation (n = 6), endovascular occlusion (n = 3), or a combined approach (n = 1). Shunt occlusion was successful in all cases. Serum ammonia decreased from 130 ± 115 μmol/L preoperatively to 31 ± 15 μmol/L postoperatively (P = .03). Additional benefits included resolution of neurocognitive dysfunction (n = 3), liver nodules (n = 1), and vaginal bleeding (n = 1).

Conclusion

Correction of type II PSS relieves a wide array of symptoms. Surgery is indicated for patients with clinically significant shunting. A refined classification system will permit future comparison of patients with similar physiology.

Section snippets

Methods

All patients treated for type II congenital PSS at Children's Memorial Hospital (Chicago, IL) from 1998 through 2009 were retrospectively reviewed. Inpatient and outpatient medical records were queried to determine demographic information, presenting symptoms, associated medical problems, operative course, and clinical outcome. The study was approved by the hospital's institutional review board. Continuous variables are reported as mean ± SD as well as median values. Comparison of continuous

Results

Ten children (7 boys) with type II congenital PSS were identified (Table 2). The median age at presentation was 5.5 years (range, 0.5-16 years). Hyperammonemia was the most frequent problem (cases 1-4, 7, 8, and 10), and the mean preoperative ammonia was 130 ± 115 μmol/L (median, 94 μmol/L; reference range, 11-35 μmol/L). Treatment of hyperammonemia with severe restriction of dietary protein consumption, lactulose, or gut antibiotic administration had been attempted in most cases. Four patients

Discussion

Review of our experience in managing children with type II congenital PSS illustrates the benefits of occluding the abnormal communication. Shunt anatomy is highly variable with resultant differences in clinical presentation and optimal operative approach. Successful shunt closure can alleviate symptoms of hyperammonemia and related neurologic symptoms, result in resolution of regenerative liver nodules, reverse growth impairment, and improve coagulopathy in affected patients.

It is increasingly

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Successful Liver Transplantation From a Deceased Donor With Congenital Extrahepatic Portosystemic Shunt: A Case Report
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Congenital extrahepatic portosystemic shunt belongs to a family of rare vascular abnormalities. We present a case of congenital extrahepatic portosystemic shunt occurring in a 42-year-old man who died of cerebral hemorrhage and donated his liver. His portal vein angiography revealed that the main portal vein communicated with the left renal vein, suggesting a portosystemic shunt. A liver biopsy showed that the liver tissue structure was normal. His liver was not involved, and the transplantation was carried out smoothly. The recipient recovered smoothly, and the function of the transplanted liver was good.
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To investigate the diagnostic and monitoring value of ultrasound (US), computed tomography angiography (CTA), and portal venography in surgical ligation of congenital extrahepatic portosystemic shunts (CEPS) in children.
We retrospectively analysed different imaging examinations of 15 children with CEPS. Development of the portal vein before shunt occlusion, shunt location, portal vein pressure, main symptoms, diameter of the main portal vein, and location of secondary thrombosis after shunt occlusion were recorded. Final classification diagnosis was made by portal venography after shunt occlusion, and consistency with other imaging examinations in diagnosing development of the portal vein was calculated using Cohen’s kappa.
Compared with portal venography after shunt occlusion, US, CTA, and portal venography before shunt occlusion had poor consistency in showing the development of hepatic portal veins (Kappa value 0.091–0.194, P > 0.05). Six cases developed portal hypertension (40–48 cmH₂O) during the temporary occlusion test, and US showed that portal veins gradually expanded after shunt ligation. Eight patients with haematochezia had inferior mesenteric vein (IMV)-iliac vein (IV) shunts. After surgery, secondary IMV thrombosis was observed in eight cases and secondary splenic vein thrombosis in four cases.
Portal venography with occlusion testing is very important to accurately evaluate the development of the portal vein in CEPS. The portal vein needs to expand gradually, and partial shunt ligation surgery is necessary in cases diagnosed as portal vein absence or hypoplasia before occlusion testing to avoid severe portal hypertension. After shunt occlusion, US is effective in monitoring portal vein expansion, and both US and CTA can be used to monitor secondary thrombi. IMV-IV shunts can cause haematochezia and are prone to secondary thrombosis after occlusion.
Congenital extrahepatic Portosystemic shunt with hypoplasia of the intrahepatic inferior vena cava: A rare case report
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Congenital portosystemic shunt (CPS) is a rare vascular anomaly resulting in diversion of splanchnic or portal blood into the systemic circulation. Other vascular malformations associated with this entity are uncommon. A 4-year-old female child with a diagnosis of acute viral hepatitis had an incidental finding of extrahepatic CPS on a Doppler abdominal ultrasound. Contrast-enhanced computed tomography showed a dilated portal vein having H-type side-to-side communication with a hypoplastic intrahepatic portion of the inferior vena cava and a prominent dilated azygos vein. There was retroaortic left renal vein drained into the IVC which was visualized in its entirety. Echocardiography findings were normal and the patient was discharged after symptomatic treatment that achieved improvement. With the expansion of abdominal imaging, incidental cases of CPS are increasingly being diagnosed in children. Although vascular malformations associated with CPS are rare, early diagnosis of cases helps avoid complications during shunt closure.
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Abernethy malformation or congenital portosystemic shunt is a rare congenital vascular malformation and anomaly of the splanchnic venous system defined by diverting portal blood away from the liver. It is commonly associated with multiple congenital anomalies. Imaging modalities such as computed tomography or magnetic resonance have a crucial role in prompting diagnosis and determining the prognosis based on the type of malformation and associated anomalies. Misdiagnosis could be harmful and may lead to inappropriate treatment. We present a case of Abernethy malformation with a complete end-to-side shunt of portal venous flow into the systemic venous flow and complete bypass of the liver, which was initially misdiagnosed with portal venous thrombosis.

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BAPS PapersManagement and classification of type II congenital portosystemic shunts☆

Abstract

Background

Methods

Results

Conclusion

Section snippets

Methods

Results

Discussion

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr

J Pediatr Surg

Gastroenterology

J Pediatr Surg

J Vasc Interv Radiol

Ann Thorac Surg

J Pediatr

J Pediatr Surg

J Pediatr Surg

J Pediatr

J Pediatr Surg

Account of two instances of uncommon formation in the viscera of the human body

Phil Trans R Soc

The clinical anatomy of congenital portosystemic venous shunts

Clin Anat

Portal-systemic encephalopathy due to a congenital extrahepatic portosystemic shunt: three cases and literature review

Hepatogastroenterology

BAPS Papers
Management and classification of type II congenital portosystemic shunts☆