Application of the “five-plane method” for the prenatal diagnosis of fetal extrahepatic portosystemic shunts: a retrospective cohort pilot study

Introduction

A congenital extrahepatic portosystemic shunt (CEPS) is a rare congenital vascular anomaly in which splanchnic venous blood bypasses the liver and drains directly into the systemic circulation through a congenital shunt (1). These alterations in the splanchnic circulation produce hemodynamic and physiological changes that can lead to severe complications, including hepatic encephalopathy, pulmonary vascular diseases (hepatopulmonary syndrome or pulmonary arterial hypertension), benign or malignant liver tumors, and biological and metabolic disorders (2-5). Early diagnosis and intervention are critical to prevent long-term complications and to improve the prognosis of affected individuals.

Postnatally, CEPS is often undetected until severe complications arise prompting further investigation. The diagnosis of CEPS via postnatal ultrasound is particularly challenging, and most cases are detected by computed tomography (CT), magnetic resonance imaging (MRI), or digital subtraction angiography (DSA). Prenatal Doppler ultrasound offers a clear advantage in the diagnosis of CEPS, but due to limited awareness of this condition, it is prone to missed diagnoses. This study aimed to present a new approach, referred to as the “five-plane method”, to improve the detection of CEPS by prenatal ultrasound. The findings of this study could inform future prenatal counseling and clinical decision making. We present this article in accordance with the STROBE reporting checklist (available at https://qims.amegroups.com/article/view/10.21037/qims-2025-1035/rc).

Methods

Study participants

Five cases of CEPS diagnosed via prenatal ultrasound at the Hunan Provincial Maternal and Child Health Care Hospital from January 2017 to December 2023 were included in this retrospective study. Among these cases, the diagnoses of the four live-born infants were confirmed through surgical findings, with follow-up periods ranging from 1 year and 3 months to 5 years post-surgery. In the pregnancy termination case, the diagnosis was confirmed by autopsy with the informed consent of the mother and the family. The study was conducted in accordance with the Declaration of Helsinki and its subsequent amendments. The study was approved by the Hunan Provincial Maternal and Child Health Care Hospital Ethics Committee (No. 2024003), and informed consent was obtained from the mothers and families of the participants.

Ultrasound instruments

This study used GE Voluson E6, E8, and E10 series color Doppler ultrasound diagnostic devices for transabdominal scanning. Both two-dimensional and three-dimensional abdominal volume probes were employed. The E10 probe operated at frequencies of 4–6 and 5–7 MHz, while the E6 and E8 probes operated at 2–5 and 4–6 MHz, respectively.

The “five-plane method” for fetal umbilical-portal vein (PV) system ultrasound

Fetuses referred to Hunan Provincial Maternal and Child Health Care Hospital for suspected malformation were scanned using the “five-plane method”, which employs high-definition flow imaging (HDFI) of the fetal abdomen across four transverse planes and one coronal plane. Each plane is labeled with different colors to clearly show the blood vessels that are easier to observe in each plane (Figure 1). The technique is demonstrated in Video 1.

Figure 1 The “five-plane method” using high-definition flow imaging for the fetal portal venous system. (A) Umbilical vein and intrahepatic portal venous system (yellow block in F). (B) Origin of the ductus venosus (red block in F). (C) Hepatic venous system (green block in F). (D) Connection between the main portal vein and the portal sinus (blue block in F). (E) Junction of the splenic vein and superior mesenteric vein with the main portal vein (purple block in F). (F) Schematic diagram of fetal liver portal-body venous scanning. ARPV, anterior right portal vein; BL, bladder; DV, ductus venosus; IVC, inferior vena cava; LHV, left hepatic vein; LPV, left portal vein; MPV, main portal vein; MHV, middle hepatic vein; PRPV, posterior right portal vein; PS, portal sinus; RHV, right hepatic vein; RPV, right portal vein; SMV, superior mesenteric vein; SP, spine; ST, stomach bubble; SV, splenic vein; UV, umbilical vein.

This study introduced an innovative technique known as the “five-plane method” to systematically evaluate the vascular connections within and outside the fetal liver.

v Plane 1: visualization of the intrahepatic portal venous system (IHPVS). Beginning with the fetal abdominal circumference measurement, the ultrasound probe is adjusted to visualize the umbilical vein (UV) as it enters the liver and connects to the sagittal part of the left branch of the PV, forming a “L-shaped” configuration at a right angle where the bend corresponds to the portal sinus (PS) (Figure 1A).

v Plane 2: visualization of the ductus venosus (DV). From Plane 1, the probe is adjusted in the cephalic direction of the fetus to reveal the DV, which continues from the sagittal part of the left branch of the PV, and drains into the proximal segment of the inferior vena cava (IVC) (Figures 1B).

v Plane 3: visualization of the hepatic venous system. The probe is further angled cephalically from Plane 1 to provide a clear view of the hepatic venous system. This includes the left, middle, and right hepatic veins converging into the IVC, offering a detailed assessment of hepatic venous drainage (Figure 1C).

v Plane 4: visualization of the confluence of the main portal vein (MPV) and the PS. Using the PS as a reference from Plane 1, the probe is slightly repositioned to trace the MPV and its confluence with the PS. This reveals the MPV extending into the right anterior and posterior branches, which transport hypoxic blood from the spleen and the gastrointestinal tract to the right liver lobe. Plane 4 enriches the observations initiated in Plane 1 by providing a detailed depiction of the MPV, along with its anterior and right posterior branches, enhancing the understanding of the architecture of the IHPVS (Figure 1D).

v Plane 5: coronal view of the first-order branches of the extrahepatic portal venous system (EHPVS). The probe is rotated 90 degrees from Plane 4 to capture a coronal view of the first-order branches of the EHPVS. This plane distinctly visualizes the merging of the superior mesenteric vein (SMV) and splenic vein (SV) into the MPV, along with their respective arteries (Figure 1E).

EHPS ultrasound classification method

The “five-plane method” detects the origins of the shunt vessels, while HDFI is used to trace the pathways of these abnormal vessels and their eventual drainage points. Additionally, spatio-temporal image correlation technology can be employed for three-dimensional reconstruction, which further enhances the diagnosis and categorization of CEPS. Kobayashi (6), a Japanese researcher, categorized CEPS into three types based on the drainage site of the shut vessels: Type A (a shunt from the PV directly into the IVC); Type B (a shunt from the PV to the renal vein); and Type C (a shunt from the PV to the iliac vein).

Fetal cardiac function assessment techniques

Throughout gestation, changes in fetal cardiac function were monitored using the cardiovascular profile score (CVPS). A score of 10 was deemed normal, while a score below 5 indicated poor perinatal outcomes (7-10). Postnatally, the diagnosis and type of CEPS were confirmed through surgical examination, radiographic studies, and/or postmortem autopsy.

Results

Prenatal characteristics

The mothers’ ages ranged from 26 to 35 years (median age: 29 years). Prenatal ultrasound examinations were performed between 24 and 32 weeks of gestation [median gestational age (GA): 30 weeks]. Among the five cases with CEPS, two were classified as Type A, two as Type B, and one as Type C. The shunt vessels in Cases 1 and 5 originated from to the PS, and were detected in Plane 1, while the shunt vessels in Cases 2, 3, and 4 originated from the main trunk of the PV, and were detected in Plane 4. Plane 5 was used to verify the origin of the abnormal blood vessels. All five cases were confirmed using this method.

The IHPVS was well developed in all five cases. However, in Case 5, the development of the SV and MPV was poor. Conversely, the other four cases displayed well-developed SVs, SMVs, and MPVs. Cardiac function monitoring via ultrasound revealed that Case 5 had global cardiac enlargement and an elevated cardiothoracic ratio of 0.45 at the 24-week mark. Case 5 also presented with a pericardial effusion measuring 0.26 cm in width, and had a CVPS score of 8. The remaining four cases exhibited only mild right ventricular hypertrophy with preserved cardiac function, and each had a CVPS score of 10. Structural abnormalities were noted in all cases: Case 5 had an increased nuchal translucency measurement, while the other four cases had normal nuchal translucency measurements. For further details, see Table 1. Doppler assessments of the umbilical artery (UA), middle cerebral artery, and DV were unremarkable across all cases. Case 5 underwent chromosomal karyotyping, which showed a 46,X,inv(Y) karyotype. The other four cases underwent Down syndrome screening, and were all classified as low risk.

Pregnancy outcomes

Postnatal evaluation confirmed complete concordance with the prenatal diagnoses in all five cases, resulting in a sensitivity and specificity of 100% (for specific postpartum details, see Table 2). Of the four live births, two were delivered via cesarean section, and the other two were delivered vaginally. The birth weight of the newborns ranged from 2.6 to 3.2 kilograms (average weight: 2.9 kilograms, standard deviation: 0.26 kilograms). Prior to surgery, all four live-born infants exhibited elevated levels of transaminases, two infants showed increased total bile acid levels, one infant had elevated total bilirubin levels, and two infants presented with decreased total protein levels. All four live-born infants received surgical intervention: Cases 1 and 4 underwent percutaneous shunt treatment, while Cases 2 and 3 underwent vascular shunt ligation (VSL).

Follow-up was conducted via telephone, with periods ranging from 1 year and 3 months to 5 years. All the live-born infants had favorable outcomes.

Imaging characteristics of CEPS (Types A, B, and C)

Type A: shunt from the PV directly into the IVC

Case 1: A 34-year-old pregnant woman at 33 weeks and 1 day of gestation showed the following specific sonographic features: An anomalous vessel originating from the PV sinus before splitting into the left and right branches. This vessel traveled behind the gastric bubble, crossed over the abdominal aorta, and drained into the IVC (Figure 2A-2C). The IVC was dilated, measuring about 0.41 cm in width (Figure 2D), and the anomalous vessel exhibited a portal venous flow pattern with a peak systolic velocity (PSV) of 20.4 cm/s (Figure 2E). The fetal heart was observed to be mildly enlarged (Figure 2F), and the IHPVS and hepatic venous system, including their tributaries, were clearly delineated (Figure 2G,2H), along with the MPV, SV, and SMV (Figure 2I). Serial ultrasound assessments of the shunt vessel and cardiac function were conducted until the neonate’s delivery at 36 weeks and 6 days of gestation.

Figure 2 Prenatal ultrasound images of Case 1. (A) High-definition flow imaging showed the shunt vessel originating from the portal vein sinus, traveling leftward, and then turning downward along the inner side of the stomach bubble (as indicated by the arrows). (B) Spatio-temporal image correlation imaging technique showed the course of the shunt vessel (as indicated by the arrows). (C) Spatio-temporal image correlation imaging technique showed the shunt vessel draining into the IVC (as indicated by the arrow). (D) Two-dimensional imaging displayed the shunt vessel draining into the IVC. (E) Doppler spectrum of the shunt vessel. (F) Two-dimensional image of the fetal heart in the four-chamber view. (G) High-definition flow imaging image of the intrahepatic portal venous system. (H) High-definition flow imaging image of the hepatic venous system. (I) High-definition flow imaging image of the extrahepatic portal vein system. AO, aorta; BL, bladder; DAO, descending aorta; DV, ductus venosus; IVC, inferior vena cava; L, left; LHV, left hepatic vein; LPV, left portal vein; MHV, middle hepatic vein; MPV, main portal vein; PS, portal sinus; PV, portal vein; R, right; RHV, right hepatic vein; RPV, right portal vein; SMV, superior mesenteric vein; SP, spine; ST, stomach bubble; SV, splenic vein; Umb-ED, umbilical end diastolic velocity; Umb-HR, umbilical heart rate; Umb-MD, umbilical artery medio-diastolic velocity; Umb-PI, umbilical pulsatility index; Umb-PS, umbilical peak systolic velocity; Umb-RI, umbilical resistive index; Umb-S/D, umbilical systolic/diastolic ratio; Umb-TAmax, umbilical time-averaged maximum velocity; UV, umbilical vein; V, anomalous shunt vessel.

The 6-month postnatal follow-up revealed that the shunt vessel was still present, along with abnormal liver function tests showing increased alanine aminotransferase (ALT) and aspartate aminotransferase (AST) levels, as well as hypoproteinemia. The infant subsequently underwent percutaneous shunt treatment at another medical facility. An indirect venography performed through the SMV showed the abnormal shunt from the PV to the IVC (Figure 3A). A vascular occluder was deployed at the site of the shunt to successfully embolize the vessel (Figure 3B). Subsequent indirect venography performed through the SMV confirmed the absence of portal-to-IVC shunting (Figure 3C). The child’s postnatal prognosis at 1 year and 6 months was favorable.

Figure 3 Portal venous indirect angiography images of Case 1. Pre-embolization (A,B) and post-embolization (C). The embolization procedure confirmed the resolution of the anomalous shunt (as indicated by the red circle and arrow, which delineate the abnormal shunting vessel). PV, portal vein; SMV, superior mesenteric vein; SV, splenic vein.

Type B: PV draining into renal vein

Case 3: A 35-year-old expectant mother at 32 weeks and 3 days of gestation underwent an ultrasound that revealed an anomalous vessel emerging from the proximal portion of the PV. This vessel extended posteriorly and to the left, then looped back, descended, and ultimately emptied into the left renal vein (LRV) (Figure 4A-4C). The fetal heart was observed to be mildly dilated (Figure 4D), and the DV, the main stem of the PV, and its intrahepatic branches were all visualized. Surveillance of the shunt vessel and cardiac function via ultrasound was maintained until the neonate’s delivery at 35 weeks and 6 days.

Figure 4 A compilation of prenatal ultrasound, postnatal ultrasound, and three-dimensional computed tomography angiography images of Case 3. (A) Prenatal high-definition flow imaging showed the shunt vessel emerging from the origin of the portal vein, tracking laterally to the left, and then curving downward along the inner aspect of the gastric bubble (as indicated by the arrows). (B) Prenatal high-definition flow imaging captured the shunt vessel draining into the left renal vein (as indicated by the arrows). (C) Spatio-temporal image correlation imaging showed the trajectory of the shunt vessel (as indicated by the arrows). (D) A two-dimensional ultrasound image showed the fetal heart in the four-chamber view. (E) Postnatal color Doppler flow imaging delineated the shunt vessel’s course (as indicated by the arrows). (F) Postnatal three-dimensional computed tomography angiography showed the shunt vessel’s path (as indicated by the arrows). AO, aorta; BL, bladder; DAO, descending aorta; DV, ductus venosus; HV, hepatic vein; IVC, inferior vena cava; LK, left kidney; LKV, left kidney vein; LPV, left portal vein; LRV, left renal vein; MHV, middle hepatic vein; PV, portal vein; RHV, right hepatic vein; RK, right kidney; RKV, right kidney vein; RPV, right portal vein; SMV, superior mesenteric vein; SP, spine; SP-V, splenic vein; ST, stomach bubble; SV, splenic vein; UV, umbilical vein.

Postnatal ultrasound confirmed the shunt vessel’s drainage into the LRV (Figure 4E). After birth Postnatal three-dimensional CT angiography demonstrated two anomalous vessels originating from the main trunk of the PV: a large shunt vessel (v1, marked by a red arrow in Figure 4F) draining into the LRV, and a smaller shunt vessel (v2, indicated by a white arrow in Figure 4F) draining into the SMV. During the 6-month postnatal follow-up, the shunt vessels persisted. Additionally, the liver function tests showed abnormalities (elevated ALT and AST levels), and the cardiac enzyme panel indicated increased levels of creatine kinase, creatine kinase-myoglobin, and myoglobin. The child underwent ligation of the shunt vessel (v1) at another medical facility and was diagnosed with CEPS, specifically Abernethy type II. The prenatal diagnosis was consistent with the surgical outcome, and the child experienced no complications postoperatively. The child’s postnatal prognosis at the 4-year, 2-month follow-up was favorable.

Type C: PV draining into iliac vein

Case 5: A 26-year-old gravida at 24 weeks and 4 days of gestation underwent an ultrasound that revealed an anomalous vessel originated from the PV sinus, then tracking posteriorly and laterally to the left hepatic region, between the liver and the gastric bubble, and descending into the pelvic area to merge with the right internal iliac vein (RIIV) (Figure 5A-5C). The venous flow in the abnormal vessel was noted to have a PSV of 21.2 cm/s (Figure 5D). The fetal heart was observed to be mildly enlarged (Figure 5E), and the DV, along with branches of the intrahepatic PV, were visualized (Figure 5F,5G), whereas the SV and the MPV were indistinct.

Figure 5 Prenatal ultrasound images of Case 5. (A) Two-dimensional imaging showed the course of the shunt vessel at its initial extrahepatic segment (as indicated by the arrows). (B) High-definition flow imaging showed the trajectory of the shunt vessel at its origin (as indicated by the arrows). (C) High-definition flow imaging illustrated the shunt vessel draining into the RIIV (as indicated by the arrows). (D) The shunt vessel exhibited a portal venous flow spectrum. (E) Two-dimensional image of the fetal heart in the four-chamber view. (F) Color Doppler flow imaging displayed the ductus venosus (as indicated by the arrow). (G) High-definition flow imaging visualized the intrahepatic portal venous system. AO, aorta; BL, bladder; DV, ductus venosus; IVC, inferior vena cava; L, left; LPV, left portal vein; PS, portal sinus; R, right; RIIV, right internal iliac vein; RPV, right portal vein; SP, spine; SP-LPV, sagittal part-left portal vein; ST, stomach bubble; UV, umbilical vein.

The pregnancy was terminated. Fetal autopsy findings revealed that the anomalous vessel, originating from the PV sinus (Figure 6A), measured approximately 2–3 mm in diameter, and descended to the left of the gastric bubble into the pelvis. It crossed the rectum and the left external iliac vessels before terminating in a connecting branch that entered the RIIV (see Figure 6B, in which the yellow arrow marks the connecting branch). This branch also extended to anastomose with the rectal venous plexus, and the RIIV was markedly dilated in comparison to the left internal iliac vein (Figure 6C). The infant showed oligodactyly with only four toes on each foot, along with syndactyly affecting some fingers and toes on the left hand and both feet. There was also partial agenesis of terminal phalanges in certain fingers and toes (Figure 6D).

Figure 6 Corresponding pathological specimen of Case 5. Shunt vessel crossed the rectum and the left external iliac vessels (indicated by white arrows) before terminating in a connecting branch that entered the RIIV (indicated by an yellow arrow). (A) The course of the initial segment of the shunt vessel, which originates from the PVS. (B) The shunt vessel gives off a communicating branch that drains into the RIIV, with this branch also sending a tributary that anastomoses with the rectal venous plexus. (C) Images of the left and RIIVs. (D) Images of the child’s hands and feet. BL, bladder; DV, ductus venosus; IVC, inferior vena cava; L, left; LEIV, left external iliac vein; LIIV, left internal iliac vein; PVS, portal vein sinus; REIV, right external iliac vein; RIIV, right internal iliac vein; ST, stomach bubble; UV, umbilical vein.

Discussion

CEPS, also known as congenital absence of the PV, is an extremely rare congenital vascular anomaly. It was first described by the British surgeon Abernethy in 1793, and is thus also referred to as Abernethy’s malformation (11). The hallmark of this condition is the direct diversion of portal venous blood into the systemic venous circulation, either bypassing the liver entirely or allowing only minimal flow through it. According to literature reports (1), CEPSs rarely close spontaneously after birth in both pediatric and adult populations. If these conditions are not identified and treated promptly, they can cause severe, irreversible complications that may threaten the patient’s life (2,12,13). Consequently, the prenatal detection of CEPS, coupled with vigilant postnatal surveillance and management, can significantly mitigate the risk of complications, ultimately enhancing the patient’s quality of life.

Numerous case reports on CEPS have been published both domestically and internationally, primarily focusing on newborns and adults (14-17), with comparatively fewer diagnoses made during the fetal stage. Achiron and Kivilevitch (3) was the first to report CEPS through prenatal ultrasound examinations, indicating that this condition can be identified and diagnosed prior to birth. The present study employed the “five-plane method” in prenatal ultrasound to successfully identify abnormal vascular origins in five CEPS cases, and evaluate the development of the fetal intrahepatic system and EHPVS. Such assessments are vital for the classification of CEPS, which significantly influences the prognostic evaluation of affected children and the formulation of treatment strategies.

This study introduced an innovative technique known as the “five-plane method” to systematically evaluate the vascular connections within and outside the fetal liver. Existing assessment methods include the “three-plane method”, which was proposed by Yagel et al. (18), and can be used for the systematic examination of fetal hepatic blood vessels, effectively visualizing the venous duct, the intrahepatic venous system, and parts of the IHPVS. However, this approach harbors fundamental anatomical blind spots in the diagnosis of portosystemic shunts. Notably, it fails to capture the connections between the UV and the intrahepatic PV, and it ignores the portal confluence where the MPV branches into the superior mesenteric and SV. To address these limitations, we introduced the novel “five-plane method”, which incorporates two transformative innovations via the addition of Planes 1 and 5. The continuous scanning of the abdominal transverse section not only reveals the pathways of the intrahepatic blood vessels but also displays the primary branches of the EHPVS. This method is straightforward and coherent, making it suitable for prenatal screening, as demonstrated in the accompanying video.

Plane 1 facilitates the evaluation of how the UV interfaces with the IHPVS post-liver entry, while Plane 4 assesses the junction between the MPV and the IHPVS. By using both Planes 1 and 4, the development of the IHPVS can be assessed, which is crucial for identifying the origins of CEPS vessels and minimizing the oversight of CEPSs. In our case series, the shunt vessels in Cases 1 and 5 were traced back to the PS, while in Cases 2, 3, and 4, they originated from the main trunk of the PV. The origins of these shunt vessels were near the main trunk of the PV. They were detected in Planes 1 and 4, as illustrated in Figures 2A,4A,5A. Achiron and Kivilevitch (3) described two prenatal cases of Abernethy II, one of which exhibited a well-developed IHPVS; however, the MPV bypassed the IHPVS, emptying directly into the IVC. Plane 4 is also instrumental in the diagnosis of such cases.

Plane 2 is consistent with the B-plane scanning method proposed by Yagel et al. (18), which can visualize the hepatic venous system. Plane 3 displays the DV, improving upon Yagel et al.’s Plane C. Initially, Plane C was designed to visualize the course of the DV in a paramedian sagittal section of the fetal upper abdomen. However, obtaining this plane can be challenging due to the influence of fetal positioning during the second and third trimesters. To improve upon this, we adapted the technique by angling the probe cephalad from the fetal Plane 1 to achieve the refined Plane 3. By appropriately adjusting the color gain, the bright regions effectively indicate the DV. This method enables us to quickly and accurately locate the DV in a transverse section of the fetal abdomen.

Plane 5 builds on Plane 4 by rotating the ultrasound probe 90° along the main trunk of the PV and tilting it appropriately. This allows for a clear view of the confluence of the SMV and the SV into the main trunk of the PV in the coronal plane of the fetal abdomen. In our study, Plane 5 was used to verify the origin of abnormal blood vessels. Plane 5 is easier to obtain before childbirth than after, as intestinal gas can interfere with its acquisition during postpartum ultrasound. Indeed, some reported cases of CEPS postpartum originate from the SMV or SV (15,19). Most of these cases have been detected using CT, MRI, or DSA (15,20), indicating that diagnosing such CEPSs via ultrasound postpartum is particularly challenging. Therefore, using Plane 5 prenatally offers a distinct advantage in detecting CEPS. Further, the identification of any abnormalities (e.g., the dilation of the IVC or the UV, the absence of the DV, or the incomplete visualization of the intrahepatic PV branches) in Planes 1 to 4 may suggest a potential CEPS. In such instances, Plane 5 can be employed for a more thorough investigation of the EHPVS. The use of Plane 5 for additional assessment may also mitigate the risk of missed diagnoses of CEPS. One large-scale cohort study (n=1,810) (18) used the three-plane method to systematically examine the venous system during 20–24 weeks of gestation, and achieved a success rate of 97.9%. As the “five-plane method” represents an extended technique based on the same underlying principles, it follows identical GA applicability, and this method can be used in the usual second-trimester morphologic surveillance. Consequently, in the current case series, all examinations were performed beyond 20 weeks of gestation.

Kobayashi (6), a Japanese researcher, further categorized this condition into three types based on the drainage site of the CEPS vessels: Type A, where the PV directly drains into the IVC; Type B, where the PV directly drains into the renal vein; and Type C, where the PV directly drains into the iliac vein. This classification system aids in evaluating potential complications. Type A frequently occurs alongside cardiac and other anomalies, and the prevalence of hepatic encephalopathy is higher in Types A and B than Type C. Individuals with Type C often experience lower gastrointestinal bleeding, possibly due to the PV shunting through the internal hemorrhoidal venous plexus, which connects to the iliac vein and establishes extensive collateral circulation. In this study, Cases 1 and 2 were classified as Type A, Cases 3 and 4 as Type B, and Case 5 as Type C. Case 1 was further complicated by a persistent left superior vena cava (PLSVC), Case 3 by a single UA, and Case 4 by a ventricular septal defect (VSD). In Case 5, the shunt vessel had a communicating branch that drained into the RIIV, which also extended a tributary that connects with the rectal venous plexus.

The “five-plane method” is crucial for identifying where abnormal blood vessels originate and for assessing the development of the IHPVS. This information is vital for choosing treatment options and evaluating the prognosis of patients with CEPS after birth. In 1994, Morgan et al. (1) introduced a classification system that categorizes CEPS into two distinct types based on hepatic perfusion patterns: Abernethy Type I, which is characterized by the malformation of the hypoplastic portal venous system, and the complete diversion of the PV to the systemic circulation with an absence of portal blood flow within the liver; and Abernethy Type II, which is characterized by partial shunting of the PV to the systemic circulation, allowing for some residual portal blood flow in the liver. Abernethy Type I is regarded as the most severe form of CEPS, with liver transplantation being the most effective treatment (21,22).

Kanazawa et al. (23) highlighted that detecting Type I CEPS using ultrasound or CT scans is difficult, primarily because the intrahepatic PVs can be exceedingly small and difficult to visualize clearly. In such cases, the development of the IHPVS often needs to be further verified through a shunt occlusion test. We believe that the prenatal diagnosis of Type I CEPS requires extreme caution, multidisciplinary consultation, and postnatal confirmation via shunt occlusion studies, which aligns with the views expressed by Jimenez-Gomez J (24).

The “five-plane method” serves only as an initial screening tool for intrahepatic portal development. If the first and fourth planes can be detected through this method, it can be preliminarily deduced that the development of the intrahepatic portal system is adequate.

In this study, all five cases were identified as Abernethy Type II. Four of these cases (i.e., Cases 1, 2, 3, and 4) were delivered naturally and subsequently underwent scheduled surgeries postnatally due to detected liver function irregularities. Cases 1 and 4 were treated by percutaneous shunt, while Cases 2 and 3 underwent ligation of the shunt vessels. All four cases had favorable prognoses, and no complications were noted, with follow-up periods ranging from 1 year and 3 months to 5 years. There is no global consensus on the treatment of Abernethy Type II. Research has demonstrated that early surgical intervention is effective in preempting the development of complications and alleviating symptoms. Surgical techniques, including surgical ligation and interventional embolization, should be tailored to each individual’s anatomical features and clinical presentation (2,25).

Case 5, which was complicated by additional severe structural deformities and chromosomal anomalies, resulted in termination of the pregnancy. For patients with isolated CEPS, the prognosis is generally favorable with planned surgical interventions. Conversely, the prognosis for patients with complex CEPS is significantly influenced by the presence and severity of accompanying malformations.

Fetal cardiac function is a key factor in determining the prognosis of CEPS. In CEPS, the fetal portal venous blood either partially or completely bypass the liver, entering the systemic circulation directly and increasing the volume load on the heart. This can result in cardiac enlargement, valvular regurgitation, and even progress to severe conditions like congestive heart failure. Therefore, it is essential to closely monitor hemodynamic changes in fetuses with CEPS throughout pregnancy. If indicators of heart failure are present and the CVPS is below 5, poor perinatal outcomes are indicated, and early delivery should be considered. In our study, four CEPS fetuses (Cases 1, 2, 3, and 4) received regular ultrasound monitoring during gestation, but showed only mild cardiac enlargement and no signs of heart failure. Cases 1, 2, and 3 were born at term without complications, while Case 4 was safely delivered at 36 weeks and 2 days.

The co-occurrence of severe structural malformations and chromosomal abnormalities significantly affects the prognosis of CEPS patients. Studies have shown a higher prevalence of trisomy 21 among children with portosystemic shunts (26,27), and CEPS is often accompanied by other structural anomalies, particularly cardiac issues, urogenital malformations, skeletal developmental disorders, and biliary atresia (26). In our study, Case 1 presented with a PLSVC, Case 4 had a VSD, and Case 5 exhibited partial deformities of the fingers and toes, with chromosomal karyotype analysis revealing 46,X,inv(Y). Therefore, when CEPS is detected via prenatal ultrasound, it is crucial to conduct a comprehensive examination to identify potential abnormalities in other systems. Additionally, parents should be informed about the possibility of chromosomal anomalies, and appropriate genetic counseling should be provided before any determination is made to rule out chromosomal anomalies.

Although all the prenatal diagnoses in these five cases were confirmed to be correct, resulting in 100% sensitivity and 100% specificity, our study’s small sample size limited our ability to include cases of Abernethy Type I and shunt vessels originating from the gastric veins, mesenteric veins, or SVs, which are all components of the EHPVS. We intend to expand our dataset of fetal CEPS cases, further explore the utility of the “five-plane method”, and conduct more comprehensive research in this field.

Conclusions

This study examined the prenatal ultrasound features of five cases of CEPS, highlighting the diagnostic value of the “five-plane method” in detecting CEPS in prenatal ultrasound. When CEPS is detected prenatally, comprehensive screening for any associated structural malformations or chromosomal irregularities is essential. This screening provides the foundation for prenatal consultation and the assessment of fetal prognosis. For ongoing pregnancies, regular ultrasound monitoring of fetal cardiac hemodynamics is critical for determining the optimal delivery timing, which can significantly improve newborn survival rates.

Acknowledgments

We are grateful to Weijuan Xiao and Zhengwen Fu for their support in the production of the five-plane video.

Footnote

Reporting Checklist: The authors have completed the STROBE reporting checklist. Available at https://qims.amegroups.com/article/view/10.21037/qims-2025-1035/rc

Data Sharing Statement: Available at https://qims.amegroups.com/article/view/10.21037/qims-2025-1035/dss

Funding: This study was supported by the Natural Science Foundation of Hunan Province, China (No. 2024JJ9330 and No. 2025JJ80105), Health Research Project of Hunan Provincial Health Commission (No. W20243133 and No. 20255991), Medical Research Foundation of Guangdong Province (No. A2024457), Hunan Provincial Hospital of Maternal and Child Health Care’s High-Level Talent Development Scheme (No. 20240130-1004).

Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://qims.amegroups.com/article/view/10.21037/qims-2025-1035/coif). The authors have no conflicts of interest to declare.

Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. The study was conducted in accordance with the Declaration of Helsinki and its subsequent amendments. The study was approved by the Hunan Provincial Maternal and Child Health Care Hospital Ethics Committee (No. 2024003) and informed consent was obtained from the mothers and families of the participants.

Open Access Statement: This is an Open Access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (CC BY-NC-ND 4.0), which permits the non-commercial replication and distribution of the article with the strict proviso that no changes or edits are made and the original work is properly cited (including links to both the formal publication through the relevant DOI and the license). See: https://creativecommons.org/licenses/by-nc-nd/4.0/.

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(English Language Editor: L. Huleatt)