Clinical findings, diagnosis and therapy of patent ductus venosus in children: a case series

Abstract

Background: Patent ductus venosus (PDV) is a rare form of congenital portosystemic shunt. Because of the diversity of clinical symptoms and insufficient knowledge of this condition, clinicians often fail to perform targeted examinations, resulting in missed diagnoses and misdiagnoses. This study summarized the clinical and radiological findings, as well as surgical methods, of PDV with the aim of improving early diagnosis and guiding treatment.

Methods: Clinical, laboratory, and radiologic data of patients with PDV were analyzed retrospectively. In all, 9 patients with PDV were included in the study (7 male, 2 female; median age 1.6 years, age range 16 days to 16.5 years).

Results: Data for all 9 patients with PDV were reviewed. The most common initial clinical presentations were jaundice and respiratory symptoms. Laboratory data revealed hypoxemia in 5 patients, hyperammonemia in 2, hyperbilirubinemia in 7, abnormal coagulation function in 6, abnormal myocardial enzymes in 4, hepatic dysfunction in 8, and renal dysfunction in 3. The direct imaging sign of PDV was a vascular structure connecting the left branch of the portal vein (LPV) to the inferior vena cava. Secondary imaging findings observed in all 9 patients were dilated right heart, pulmonary artery, and LPV, and an atrophic right branch of the portal vein. The main portal vein was dilated in 8 patients and shrunk in 1. Moreover, 8 patients had enlarged livers, and 3 presented with hypoperfusion in the right lobe of the liver. The spleen was enlarged in 8 patients but shrunk in 1. Renal imaging was abnormal in 2 patients. Hepatic encephalopathy was found in 4 patients; 7 patients had PDV combined with other malformations, with congenital heart disease and vascular abnormalities being the most common; 3 patients successfully underwent surgical ligation of PDV.

Conclusions: PDV can lead to multisystem damage. Secondary radiological signs of PDV play an important role in early diagnosis and preoperative evaluation. Complications and coexisting malformations were common and should not be missed during preoperative evaluation. Early surgical closure for PDV is recommended.

Keywords: Children; case series; imaging; patent ductus venosus (PDV); therapy.

Figure 1

Imaging findings in patient 3. (A) Axial CT showed a PDV (arrowhead). (B) Sagittal CT showed a PDV (arrowhead) between the dilated LPV (long arrow) and the IVC (short arrow). (C) An atrophic RPV (arrow) was detected. (D) Color Doppler ultrasound demonstrated that the blood within PDV (black arrow) flow towards the IVC (white arrowhead) from the dilated LPV (white arrow). Red indicates flow towards the probe; blue indicates flow away from the probe. (E) Multiple nodules were detected in the liver. CT, computed tomography; PDV, patent ductus venosus; LPV, left branch of the portal vein; IVC, inferior vena cava; RPV, right branch of the portal vein.

Figure 2

Imaging findings in patient 7. (A) Axial CT showed a PDV (arrow). (B) Coronal MIP CT showed a PDV (arrow) and the dilated pulmonary artery (arrowhead). (C) A PDV (arrow) was shown on VR imaging. (D) An atrophic RPV (arrow) was detected. (E) The dilated right atrium (arrowhead) and ventricle (arrow) were observed. (F) Symmetrical hyperintensity in the bilateral lenticular nucleus (arrow) was found on T1-weighted magnetic resonance imaging of the brain. CT, computed tomography; PDV, patent ductus venosus; MIP, maximum intensity projection; VR, volume rendering; RPV, right branch of the portal vein.