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Published on: May 7, 2015
Outcome of intrahepatic portosystemic shunt diagnosed prenatally
Bérengère Francois1, Fréderic Gottrand2, Alain Lachaux3
1Service de réanimation néonatale du CHU de Nice, Nice, France. beaink@gmail.com.
Insights
Congenital portosystemic shunts diagnosed before birth typically have a good outcome, with most cases resolving spontaneously within two years. This study highlights the positive prognosis for antenatal diagnoses of intrahepatic shunts.
Area of Science:
- Pediatric Surgery
- Fetal Medicine
- Vascular Anomalies
Background:
- Congenital portosystemic shunts (CPSS) are rare vascular malformations.
- Existing research often focuses on postnatal diagnoses, leaving a gap in understanding antenatal cases.
- Perinatal care organization for these patients requires specific consideration.
Purpose of the Study:
- To analyze the characteristics of infants diagnosed with congenital portosystemic shunt antenatally.
- To evaluate the organization of perinatal care for this population.
- To assess the outcomes of intrahepatic CPSS diagnosed prenatally.
Main Methods:
- Multicenter retrospective study.
- Inclusion of all patients with a prenatal diagnosis of CPSS between 1999 and 2015.
- Analysis of patient characteristics, diagnosis timing, associated conditions, and treatment outcomes.
Main Results:
- 12 patients with antenatal diagnosis of CPSS were included.
- Prenatal diagnosis occurred at a median of 26.5 weeks gestation.
- All cases were intrahepatic CPSS; 3 had congenital cardiopathy, 1 had Bannayan-Zonana syndrome.
- 10 patients had favorable outcomes with conservative management, 8 showing spontaneous closure within 2 years.
- One surgical treatment failure led to focal nodular hyperplasia; one interventional closure complicated by portal vein thrombosis.
Conclusions:
- Intrahepatic portosystemic shunts diagnosed prenatally generally have a favorable outcome.
- Spontaneous closure is common within the first two years of life.
- While rare, complications can occur with surgical or interventional treatments.
Abstract:
We analyzed the characteristics of the population with congenital portosystemic shunt diagnosed during the antenatal period and the organization of their perinatal care. This multicentric retrospective study included all the patients with a prenatal diagnosis of congenital portosystemic shunt. Between 1999 and 2015, 12 patients were included. Prenatal diagnosis was done at a median 26.5 weeks of gestation (21-34). All the patients presented intrahepatic CPSS, three of them had associated congenital cardiopathy, and one a Bannayan-Zonana syndrome. Ten patients had simple outcome on conservative treatment, eight of them having a spontaneous closure of their portosystemic shunt within the first 2 years of life. One patient had surgical treatment which failed and he developed a focal nodular hyperplasia. Another patient had radiological interventional closure of his shunt which was complicated by a venal portal thrombosis.
Conclusion:
Outcome of intrahepatic portosystemic shunt diagnosed prenatally is good in the majority of cases. What is known: • Multiples studies exist on congenital porto systemic shunt but when the diagnosis is done after birth. • The evolution, management, and complication are well known. What is new: • There is very few studies with only patients diagnosed in antenatal and it is a large series of cases. • Outcome of intrahepatic portosystemic shunt diagnosed prenatally is good in the majority of cases.
