Journal of Obstetrics and Gynaecology, August 2011; 31: 542–543 © 2011 Informa UK, Ltd. ISSN 0144-3615 print/ISSN 1364-6893 online
OBSTETRICS CASE REPORT
Prenatal diagnosis of extrahepatic umbilicoportosystemic shunt: impact on postnatal management G. N. Bekdache1, M. A. Hamdan2, M. A. Begam1, F. Chedid2, M. M. Tamim2 & H. Mirghani1,3
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1Fetal Medicine Unit, Department of Obstetrics and Gynecology, 2Department of Pediatrics, Tawam Hospital – Johns Hopkins Affiliated and 3Department of Obstetrics and Gynecology, Faculty of Medicine and Health Sciences, United Arab Emirates University, Al Ain, United Arab Emirates
DOI: 10.3109/01443615.2011.580395 Correspondence: G. N. Bekdache, Fetal Medicine Unit, Department of Obstetrics and Gynecology, Tawam Hospital – Johns Hopkins Affiliated, Al Ain, United Arab Emirates, PO Box 15258, Al-Ain, United Arab Emirates (UAE). E-mail:
[email protected]
Introduction Congenital agenesis of the portal venous system (CAPVS) with extrahepatic umbilicoportosystemic (EHUPS) shunt is an extremely rare abnormality that permits the diversion of the portal flow to the systemic circulation. The absence of any flow regulation (absent ductus venosus) results in increased volume and pressure in the systemic system which may compromise the haemodynamic stability of the fetus and newborn (Gorincour et al. 2005). Correct prenatal diagnosis promotes early neonatal management and decreases morbidity. We present a case of prenatal diagnosis of EHUPS, which was confirmed and managed postnatally.
Case report A 28-year-old woman G6P3A2 was referred to our Fetal Medicine Unit at 33 weeks for polyhydramnios and suspected fetal anomalies. At an outside institution, a routine anomaly scan was performed at 20 weeks and was normal. A 100 g oral glucose tolerance test was normal. US examination showed a live singleton fetus, polyhydramnios and dilated intra-abdominal segment of the umbilical vein (UV) 1.2 cm (normal 8 mm at term) (Vignal 2001). The ductus venosus (DV) was absent, and the UV drained directly into a dilated inferior vena cava (IVC) (Figure 1). The IVC measured 7 mm (normal for gestational age 4 ⫾ 1 mm; Z-score: 2.9) (Firpo et al. 2001). Fetal echocardiogram showed a dilated right atrium (RA), moderate tricuspid regurgitation (TR), normal ventricular function (shortening fraction 32%), and no pericardial effusion. RA width and length in the 4-chamber view was 2.1 and 2.2 cm, respectively (Z-scores: 2.7and 2.0, respectively) (Firpo et al. 2001). The fetus had also cardiomegaly (cardiothoracic ratio 72%) and hepatomegaly. Liver length was 7.4 cm above normal range for gestational age (Vintzileos et al. 1985). No other associated anomalies explaining the polyhydramnios were identified. Therapeutic amnioreduction was performed to relieve the patient’s symptoms. A 19G needle was used with suction. A total of 1.2 litres of amniotic fluid was drained and fetal karyotype showed a normal female (46, XX). Spontaneous vaginal delivery at 35 weeks ⫹ 5 days resulted in a live female weighing 3,645 g. Apgar scores were 5 at 5 min and 10 at 10 min. After birth, the patient had mild respiratory distress.
Echocardiogram confirmed normal cardiac structure and function, dilated RA and right ventricle (RV), and mild pericardial effusion (Z-scores of RA width and RV end-diastolic dimension were 2.8 and 2.6, respectively) (Kampmann et al. 2000). Chest radiogram revealed cardiomegaly with increased pulmonary vascular markings. The cardiothoracic ration was 75%. Laboratory evaluation showed hypoalbuminaemia (albumin level 23 g/l), hypoglycaemia, total and direct hyperbilirubinaemia (total bilirubin level was 97 μmol/l) and elevated gamma-glutamyl transferase enzyme (380 IU/l). Because of the clinical, laboratory and ultrasound abnormalities, it was decided to attempt transcatheter embolisation of the shunt via the UV. Angiography through the UV confirmed the absence of DV. The portal vein connected to the IVC via a venous channel (EHUPS) (Figure 2A). This was successfully embolised using an 8 mm Amplatzer Vascular Plug® (Figure 2B). Angiography following embolisation confirmed near-complete cessation of the shunt flow, increased flow into the portal venous branches, reduction of the IVC diameter to 5.5 mm from 7.6 mm before embolisation (normal 5.2 ⫾ 0.5 mm) (Firpo et al. 2001), suggesting decreased shunt flow to the RA. The patient’s symptoms improved after 48 h, and her laboratory abnormalities resolved after 5 days. She was discharged home 5 days after the procedure in good condition. Follow-up hepatic US and echocardiogram after 1 and 6 months showed normal portal venous flow, no portosystemic shunt and normalisation of the RA and RV dimensions.
Discussion CAPVS, also known as Abernethy malformation, is a group of anomalies first reported by John Abernethy in 1793. They are classified into two types. Type I has a female predominance, and occurs when the entire portal venous supply drains into the IVC with absent intrahepatic PV, as in the case of our patient. In type II, the portal vein drains partially into the IVC through portohepatic shunts (Abernethy 1793). In type I, the associated agenesis of the DV is most likely due to low intrahepatic vascular pressure caused by the extrahepatic portosystemic shunt
Figure 1. Absent ductus venosus with the umbilical vein (UMB V) draining directly into a dilated inferior vena cava (IVC). RA, right atrium.
Obstetrics case report
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Figure 2. Angiography through the umbilical showing large venous connection between left portal vein and inferior vena cava (IVC). (A) Before, and (B) after embolisation. Flow in the IVC is significantly reduced.
(PSS) (Abernethy 1793). This results in blood diversion from the DV into the shunt. A recent classification according to the portosystemic shunt was reported by Kobayashi in 2010. Portal blood flow draining directly in the inferior vena cava was classified as type A and complicated with cardiac anomalies (Kobayashi et al. 2010). As such, careful assessment of the venous system in the standard abdominal circumference transverse plane is essential to make the diagnosis of umbilicoporto-systemic shunt prenatally. From the cord insertion at the centre of the abdomen, the left portal vein runs obliquely upwards to the liver at about a 45⬚ angle in normal conditions. Failure to visualise the portal vein with dilatation of the umbilical vein and IVC should raise suspicion. In our patient, the fetal US at 33 weeks showed dilatation of the UV and IVC, and high velocity pulsatile flow of UV (resembling that of DV) in the absence of DV, similar to that reported by VenkatRaman et al. (2002). Our patient had polyhydramnios and hepatomegaly, indicating liver congestion and excessive cardiac preload, which can lead to fetal or neonatal demise (Contratti et al. 2001; Gorincour et al. 2005; Tordjeman et al. 1996). Although agenesis of the DV is associated with fetal malformation and aneuploidy (Berg et al. 2006; Acherman et al. 2007), our patient had a normal karyotype, and no other anomalies. In the absence of additional fetal anomalies, the prognosis will depend mainly on the presence and extent of fetal congestive heart failure (CHF) (Berg et al. 2006; Acherman et al. 2007). Hence, fetuses with PSS must be followed closely for signs of CHF, including cardiomegaly, TR, effusion and hydrops, in which case early delivery should be considered. Despite having fetal cardiomegaly, dilated right heart and TR, our patient had no evidence of hydrops throughout pregnancy, until spontaneous delivery ensued at around 36 weeks. After birth, neonates with PSS can develop several complications, including worsening CHF, pulmonary hypertension, jaundice, elevated liver enzymes, progressive encephalopathy and liver tumours (Acherman et al. 2007; Uchino et al. 1999; Achiron et al. 2009; Sista and Filly 2009). Therefore, treatment should be attempted early to avoid long-term complications. Our patient had significant cardiac decompensation and hepatic dysfunction, which resolved after treatment. Trans-umbilical interventional
treatment provided a feasible and safer approach in our patient, than would surgery have offered (Uchino et al. 1999). Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.
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