Congenital Intrahepatic Portosystemic Shunt Research Articles

Case Report: Management of a congenital intrahepatic portosystemic shunt with portal vein aneurysm in a child using 3D computer-assisted partial right hepatectomy

Congenital portosystemic shunts (CPSS) are rare pediatric vascular malformations characterized by abnormal development of the portal vein, which is attributed to incomplete embryonic remodeling of the hepatic and surrounding vasculature. CPSS manifests in two main forms: intrahepatic and extrahepatic. This study details the management of a pediatric patient diagnosed with Congenital Intrahepatic Portosystemic Shunt (CIPS) who was referred to our institution. By using a computer-assisted surgical system, the right hepatectomy was successfully performed, guided by precise intraoperative navigation based on three-dimensional reconstructions of enhanced CT imagery. The patient exhibited a favorable postoperative recovery trajectory, with the absence of complications or recurrence throughout the monitoring period.

Abernethy syndrome as a cause of liver transplantation.

Congenital portosystemic shunts are rare abnormalities in which blood flow from the liver is diverted to the systemic circulation. We would like to present the case of a 48-year-old male, during his neurological follow-up he was diagnosed with a congenital intrahepatic portosystemic shunt. Embolization of the portosystemic communicating veins was attempted on two occasions, but without success. Due to the poor clinical course, the patient was presented to our transplant committee and a liver transplant was decided upon.

Imaging features, classification and clinical features of intrahepatic congenital portosystemic shunts.

Congenital portosystemic shunts (CPSS) are a rare developmental anomaly diverting blood flow from the portal venous system and the liver to the systemic venous system. This case series examines the sonographic imaging findings, shunt classification, ultrasound shunt ratios, and outcomes in nine children (5 females, 4 males) admitted to our institution between 2015 and 2022 were included in this study. The shunts were initially categorized by the Parks classification and were followed by serial ultrasounds. Clinical presentation, clinical course, laboratory data, shunt ratios, and time to shunt closure were all followed on subsequent ultrasounds. The most common type of CPPS was the Type 3 shunt. In cases where shunt ratios were measured, the shunt ratio gradually decreased in tandem with decreasing ammonia levels until spontaneous closure was achieved. Predictors of lack of shunt closure included high shunt ratios and Type 4 shunts. Patients with CPPS can be followed with the shunt ratio calculation obtained from sonographic imaging, which may correlate to ammonia levels and indicate risk of hepatic encephalopathy as well as predict speed and timing of closure.

Comparison of hematologic variables between dogs with congenital intrahepatic and extrahepatic portosystemic shunts.

Dogs with congenital intrahepatic portosystemic shunt (IHPSS) are predisposed to gastrointestinal inflammation, ulceration, and bleeding, unlike dogs with congenital extrahepatic portosystemic shunt (EHPSS). Limited information is available about hematologic differences between dogs with IHPSS and dogs with EHPSS. Compare hemogram variables between dogs with IHPSS and EHPSS. We hypothesized that hematologic variables would differ between the 2 populations, with a higher frequency and severity of anemia and microcytosis in dogs with IHPSS. Twenty-six client-owned dogs with IHPSS and 35 client-owned dogs with EHPSS. Retrospective cross-sectional study. Dogs were included if a CBC was performed before shunt attenuation. Contingency analysis was performed to determine if the frequency of clinical signs and of hematologic variables below the reference range differed between groups. Hematologic and selected biochemical variables were compared between groups using an analysis of covariance with age as a covariate. Gastrointestinal clinical signs (IHPSS, 81% vs EHPSS, 34%; P = .01), anemia (31% vs 6%; P = .01), microcytosis (77% vs 29%; P = .002), and hypochromia (77% vs 49%; P = .03) were more common in dogs with IHPSS than in dogs with EHPSS. Dogs with IHPSS had lower packed cell volume (34% vs 41%, P = .04), hemoglobin concentration (11.5 g/dL vs 13.7 g/dL, P = .03), mean corpuscular volume (57 fL vs 65 fL; P = .001), and mean corpuscular hemoglobin concentration (32 g/dL vs 33 g/dL; P = .04) than dogs with EHPSS. Dogs with IHPSS had a higher frequency of anemia, microcytosis, and hypochromia and exhibited more gastrointestinal clinical signs.

Outcome of dogs diagnosed with concurrent intrahepatic portosystemic shunts and vertebral lesions: six cases (2016-2022).

To describe the clinical outcome of dogs diagnosed with concurrent discospondylitis/vertebral physitis and congenital intrahepatic portosystemic shunts. Medical records from two academic institutions were searched for dogs diagnosed with discospondylitis and/or vertebral physitis, and a concurrent intrahepatic portosystemic shunt. Dogs were excluded if they did not undergo attenuation of their shunt, did not have a single congenital intrahepatic shunt and did not have at least 90 days of follow-up. Six dogs met the inclusion criteria and were included in the study. Discospondylitis alone was diagnosed in four dogs, vertebral physitis alone in one dog and both discospondylitis and vertebral physitis in one dog. Three dogs had a right divisional intrahepatic portosystemic shunt, and three dogs had a left divisional intrahepatic portosystemic shunt. Median duration of antimicrobial therapy was 112 days (range 14 to 240 days). Clinical resolution of discospondylitis and vertebral physitis was noted in all dogs. Endovascular attenuation was performed in all dogs a median of 82 days after presentation (range 1 to 317 days). No perioperative or postoperative complications occurred. All dogs were alive at the last available follow-up a median of 513 days after presentation (range 224 to 1504 days) and free of clinical signs associated with discospondylitis or vertebral physitis, as well as their portosystemic shunt. Dogs with intrahepatic portosystemic shunts may concurrently develop discospondylitis and vertebral physitis. With antimicrobial therapy and endovascular embolisation of their portosystemic shunt, all dogs in this study had a good outcome with clinical resolution of both disease processes. However, long-term follow-up was not obtained in all cases.

EP10.16: Prenatal ultrasonographic features and outcomes of 19 cases of congenital intrahepatic portosystemic venous shunts

EP10.16: Prenatal ultrasonographic features and outcomes of 19 cases of congenital intrahepatic portosystemic venous shunts

Canine intrahepatic portosystemic shunts: Interlobar and intralobar classifications.

This two-part study design showed that a canine congenital intrahepatic portosystemic shunt (IPSS) may be classified by its location within a liver fissure (interlobar) or lobe (intralobar). A prospective anatomic study reviewed normal canine liver morphology and showed the CT angiography (CTA) appearance of the normal canine ductus venosus (DV), which was confirmed via dissection and literature review to be between the papillary process and left-lateral liver lobe (in the fissure for ligamentum venosum). A retrospective multi-institutional case series documented the frequency of imaging findings in 56 dogs with a single IPSS that underwent portal CTA at Cornell University or the Schwarzman Animal Medical Center between June 2008 and August 2022. An interlobar IPSS was seen in 24 of 56 (43%) dogs, all arose from the left portal branch except one. These shunts were typically near the median plane, remained interlobar throughout the course, and were nearly always (96%) craniodorsal to the porta hepatis. Four types were distinguished: patent DV (11 dogs), left interlobar (11 dogs), right interlobar (1 dog), and ventral interlobar (1 dog). Only about half (46%) were in the fissure for ligamentum venosum and therefore classified as a patent DV. An intralobar IPSS was seen in 32 of 56 (57%) dogs, most (88%) originated from the right portal branch and were in the right-lateral liver lobe (21 dogs) or caudate process (7 dogs). During canine portal CTA, documenting the interlobar or intralobar location of an IPSS might increase the consistency and validity of IPSS description.

Prenatal ultrasonographic features and follow-up outcomes of 19 cases of congenital intrahepatic portosystemic venous shunts diagnosed during the foetal period

BackgroundTo investigate the prenatal ultrasonographic features and case characteristics of the congenital intrahepatic portosystemic venous shunt (IHPSS) diagnosed during the foetal period and analyse its prognosis.MethodsWe conducted a retrospective cohort study of patients diagnosed with IHPSS between 2016 and 2021. IHPSS was defined as an abnormal connection between the foetal intrahepatic portal and the hepatic veins.ResultsIn this study, 19 foetuses were identified, including 12 cases of single shunt and 7 cases of multiple shunts, with a gestational age of 33.8 ± 4.5 (range 25–40) weeks at diagnosis. In the single-shunt group, the origin position of the shunts was all from the left branch of the portal vein (LPV), whereas in the multiple-shunt group, the origin position of the shunts was from the LPV in six cases. Common concomitant intrauterine abnormalities of IHPSS include foetal growth restriction (47.4%) and foetal cardiac enlargement (21.1%). The postnatal manifestations of IHPSS include biochemical abnormalities (increased gamma-glutamyl transferase and bilirubin levels), neonatal hypoglycaemia, neonatal hyperammonaemia, pulmonary hypertension, multiple intrahepatic hyperechoic nodules, and cutaneous haemangiomas. Spontaneous closure of shunts occurred in ten cases, and the mean time to shunt closure was 8.1 months (1–28 months).ConclusionsMost IHPSS found during the foetal period is located in the left branch of the portal vein, and the gestational age at diagnosis is usually in the late second or third trimester. Spontaneous closure of shunts can occur in most live births, and the prognosis is good.

Percutaneous transvenous coil embolization of congenital intrahepatic portosystemic shunts in small- and toy-breed dogs: 20 cases (2015-2021).

To describe outcomes of small- and toy-breed dogs with a congenital intrahepatic portosystemic shunt (IHPSS) treated with percutaneous transvenous coil embolization (PTCE). 20 small- and toy-breed dogs with an IHPSS. All dogs underwent CT angiography for shunt evaluation as well as PTCE. Medical records were reviewed for pertinent data, and owners and primary veterinarians were contacted for long-term follow-up information. Dogs ranged from 1.5 to 10.0 kg (mean ± SD, 6.32 ± 2.57 kg) in weight. The equipment used to perform PTCE tended to be smaller than that previously described for larger breed dogs. Intra- and postoperative complication rates were 20% (4/20) and 5% (1/20), respectively, and included hypotension, bradycardia, hypercapnia, ventricular premature contractions, hypothermia, and regurgitation. Dogs were discharged a median of 3 days (range, 1 to 3 days) after surgery, and all dogs survived to discharge. Clinical signs resolved in 95% (19/20) of the dogs a median of 21 days after the procedure. One- and 2-year survival rates were 92%. Three dogs had died by the time of data collection; 2 of these dogs died of causes related to the IHPSS 267 and 1,178 days, respectively, after the procedure. Percutaneous transvenous coil embolization was a safe and effective option for treatment of IHPSS in small- and toy-breed dogs and offered a minimally invasive alternative to open surgical techniques. Complication and survival rates in this cohort were similar to or better than those reported in previous studies evaluating PTCE and open surgical techniques for treatment of IHPSS in dogs.

Asymptomatic congenital intrahepatic portosystemic shunt

Spontaneous asymptomatic intrahepatic portosystemic shunt is a rare anomaly of the hepatic vasculature. The main clinical manifestations may be encephalopathy, bleeding or hyperinsulinism, due to the constant shedding of blood. This article presents a case of an asymptomatic congenital intrahepatic portosystemic shunt, found in patient I., aged 25, with the results of various imaging methods, as well as an analysis of the main types of the described vascular anomaly.

Surgical treatment and outcome of intrahepatic shunts in 12 cats.

The objective of this case series was to describe the presentation, surgical treatment and outcome of a congenital intrahepatic portosystemic shunt (IHPSS) in 12 cats. A retrospective case series of cats undergoing surgical treatment for an IHPSS was undertaken. Signalment, clinical signs, imaging, surgical treatment, complications and short-term outcome (<30 days) were obtained using medical records. Long-term outcome (>1 year after first surgery) was obtained, where possible, using a health-related quality of life owner questionnaire. Seven cats were diagnosed with a left divisional shunt, three with a central divisional shunt and two with a right divisional shunt using intraoperative mesenteric portovenography. Three cats tolerated complete acute suture attenuation, eight cats underwent partial suture attenuation, four of which received complete suture ligation at a second surgery, and one cat underwent partial attenuation with a thin film band. Six cats (50%) developed post-attenuation neurological signs (PANS) after first surgery and two cats (17%) died or were euthanased due to severe PANS. Long-term outcome was available for eight cats (67%), with a median follow-up time of 1743 days (range 364-2228), and was described as excellent in five cats (63%), fair in two cats (25%) and poor in one cat (12%). Few papers exist that describe the presentation, intraoperative imaging, treatment and outcome of IHPSSs in cats. This is the first to describe surgical attenuation with a thin film band in a cat with an IHPSS. This case series reports excellent long-term outcomes in a majority of surgically treated cats with IHPSS.

Presentation, Management, and Outcome of Congenital Portosystemic Shunts in Children: The Boston Children's Hospital Experience.

Congenital portosystemic shunts (CPSS) are rare vascular malformations. We describe presentations, complications, associations, and outcomes of CPSS at Boston Children's Hospital (BCH). This was a retrospective review of children with CPSS at BCH from 2000 to 2020. Twenty-nine patients had CPSS (17 girls): 14 extrahepatic (EH) and 15 intrahepatic (IH). At diagnosis, 15 were ≤5 days, 7 <1 year, and 7 >1 year (range 1-19). Median follow-up duration was 5.2 years (interquartile range [IQR] 1.6-10.9) in EH and 2.2 years (0.2-4.2) in IH CPSS. The most common presentation was antenatal ultrasound 13 (45%) followed by hyperammonemia 10 (34%), whereas 6 (21%) were asymptomatic. Complications were noted in 17 (12/14 EH vs 6/15 IH, P = 0.008). Associated anomalies were present in 25 (14/14 EH vs 11/15 IH, P = 0.10). Spontaneous closure was observed in 8 (28%) patients with IH CPSS, all <12 months of age. Ten patients underwent shunt closure 3 (30%) by interventional radiology (IR) and 5 (50%) by surgery, whereas 2 (20%) required both. After therapeutic closure; 8 had improvement, 1 had portal hypertension, and 1 had sepsis and thrombosis. The remaining 11 patients, 8 (42%) were followed without closure: 6 of 8 (75%) EH versus 2 of 11 (18%) IH ( P = 0.02), 2 lost follow-up and 1 with complicated EH CPSS died, unsuitable for therapeutic closure. CPSS may be asymptomatic or present with complications. Spontaneous closure of IH shunts may occur in infancy, thus therapeutic closure may be deferred until age ≥ 2 years. IR and surgical closure of CPSS are associated with improvement in the majority of cases.

Fusobacterium necrophorum Infection in a Healthy 24-Year-Old Man With Congenital Intrahepatic Portosystemic Shunt

Fusobacterium necrophorum Infection in a Healthy 24-Year-Old Man With Congenital Intrahepatic Portosystemic Shunt

Intrahepatic venous collaterals in dogs with congenital intrahepatic portosystemic shunts are associated with focal shunt or hepatic vein narrowing

Dogs with congenital intrahepatic portosystemic shunts (IHPSS) occasionally have multiple smaller intrahepatic, tortuous blood vessels surrounding the primary shunt. This study was a retrospective, observational design that was also descriptive and anatomic in nature. Objectives were to characterize vascular morphology in IHPSS dogs presenting with intrahepatic venous collaterals (IVCs) relative to IHPSS dogs without IVCs, and to propose reasons for IVC development. The authors hypothesized that (a) IVCs develop secondary to flow resistance around a focal area of a shunt or draining hepatic vein narrowing and (b) the presence of IVC is associated with portal vessel development before intervention. Anonymized CT angiograms (CTA) and fluoroscopic portovenograms (FPV) of dogs with IHPSS were evaluated for the presence of IVCs, focal narrowing within the IHPSS, and intrahepatic portal vessels>5mm long. Eleven of 47 (23%) dogs had IVCs identified. IVCs were significantly associated with focal narrowing in the shunt or draining hepatic vein on CTA (P=0.039) and FPV (P=0.021). IVCs were not associated with the presence of intrahepatic portal branches>5mm long on portovenography (P=0.42) or CTA (P=0.49). Focal narrowing in the shunt (circumferential soft tissue narrowing>20% of the shunt diameter) was significantly associated with intrahepatic portal branches>5mm long on both modalities (P<0.001). IVCs are associated with focal narrowing of the shunt or draining hepatic vein in dogs with IHPSS. IVC should be distinguished from other conditions when evaluating a CTA for canine IHPSS.

Protein C and comparative biochemical changes in dogs treated with percutaneous transvenous coil embolization of congenital intrahepatic portosystemic shunts.

To evaluate protein C (PC) activity after intrahepatic portosystemic shunt (IHPSS) percutaneous transvenous coil embolization (PTCE) in dogs; to identify if PC is associated with clinical status after intervention, and to compare PC with standard biochemical values. Retrospective case series. Forty-seven client-owned dogs with IHPSS undergoing PTCE. Records were reviewed for preoperative and postoperative PC, hematocrit (HCT), mean corpuscular volume (MCV), albumin (ALB), and blood urea nitrogen (BUN). Ultimate clinical status was classified as excellent, fair, or poor, based on ongoing medical management and the presence of clinical signs. Intrahepatic portosystemic shunt was considered to be completely or incompletely occluded intraoperatively based on angiography. Postoperative PC activity increased in 37/47 (78.7%) dogs with a mean increase of 38.7% ± 2.1%. Ultimate postoperative clinical status was excellent in 16/43 (37.2%), fair in 19/43 (44.2%), and poor in 8/43 dogs (18.6%). No association was detected between preoperative PC (46.8% ± 1.8%) and ultimate clinical status but mean postoperative PC (75.7% ± 1.4%), HCT, MCV, ALB, and BUN were higher in dogs with excellent clinical status. Postoperative PC activity was higher when shunts were completely occluded (96.3% ± 10.9%), which was a finding associated with excellent status. Postoperative, but not preoperative, PC activity was higher in dogs with better ultimate clinical status. Similar trends were noted in standard hematological and biochemical values. Complete occlusion of shunts was associated with a higher postoperative PC and superior ultimate clinical status. Postoperative PC may provide valuable information about the success of PTCE for IHPSS as it relates to the ultimate status and the need for additional procedures.

Congenital anterior radial head dislocation with valgus deformity: A case report

Congenital radial head dislocation is a rare anomaly of the pediatric musculoskeletal system. The most common type is posterior, with anterior and lateral dislocations being less common. Unilateral cases are even more uncommon and were earlier thought to be non-existent. Our case report describes a young girl with unilateral congenital anterior radial head dislocation with progressive development of cubitus valgus deformity, which is a very rare occurrence. On reviewing literature, only few such cases have been described. Our patient also had a history of developmental dysplasia of hip and a congenital intra-hepatic porto-systemic shunt, which in addition to the morphological appearances of the radial head and capitellum, suggested a congenital etiology.

Characterization and natural history of congenital intrahepatic portosystemic shunts.

Congenital intrahepatic portosystemic shunts are rare vascular malformations in which abnormal communications are created between the portal veins and the hepatic veins or the inferior vena cava system. Diagnosis is made by prenatal or postpartum ultrasound. Published data regarding presentation, symptoms, and prognosis is scarce. This study aimed to better understand the natural history and the course of the intrahepatic portosystemic shunts. Data were collected from children in two medical centers who were diagnosed with congenital intrahepatic portosystemic shunts on either prenatal or postnatal sonographic screening. The subjects' medical information was collected including demographics, medical background, and sonographic and clinical outcome. Blood test results including ammonia levels and liver function tests were documented, as well as the sonographic dimensions of the shunt vessels and the spleen size. The data were analyzed using various statistical methods. Twenty-three children with portosystemic shunts were found and reviewed. Eight children were excluded from the study since records and follow-up were insufficient. Fifteen patients were included in the study (six females). All had intrahepatic shunt diagnosed either by prenatal screening or postnatal abdominal ultrasound and had more than one ultrasound and repeated blood tests. Shunt closure was observed in all children within a mean of 114.31 ± 115.05 days (median 84). There was no correlation between liver enzymes, ammonia, and ultrasound vascular and splenic diameters to time to closure. None of the children had any hepatic or other sequelae.Conclusions: Our study suggests that congenital intrahepatic portosystemic shunt is a benign, self-limiting condition in which no correlation between the size of the shunt and the blood ammonia level to the outcome of the shunt was found. This is the first study that correlated radiological measures to the outcome. These results suggest that the treating physician should reassure families and conduct minimal follow-up and interventions in children with such conditions. Further, larger and prospective studies should be done to corroborate these conclusions. What is Known: • Characteristics and natural history of intrahepatic portosystemic shunts are less defined. • The natural course of the intrahepatic malformations varies, but spontaneous, self-resolution of small shunts, usually occureswithin 1 to 2 years. What is New: • In this study, congenital intrahepatic portosystemic shunt was shown to be benign, self-limiting condition in which all shunts closed within 3 months. • No correlation between the size of the shunt and the blood ammonia level to the outcome of the shunt was found.

Congenital portosystemic shunt occlusion with an Amplatzer PFO occlusion device: a case report

BackgroundCongenital portosystemic shunts are embryological malformations in which portal venous flow is diverted to the systemic circulation. High morbidity and mortality are seen in patients with concurrent hepatic encephalopathy, hepatopulmonary syndrome, and pulmonary hypertension. Endovascular therapy, in the correct patient population, offers a less invasive method of treatment with rapid relief of symptoms.Case presentationIn this report, we discuss the treatment of a two-year-old male with abnormal chorea-like movements, altered mental status, anisocoria and hyperammonemia diagnosed with an intrahepatic congenital portosystemic shunt between the inferior vena cava and right portal vein. Given the patient’s amenable anatomy and shunt type, embolization was performed with an 18 mm Amplatzer patent foramen ovale occlusion device.ConclusionsPortosystemic shunts are a rare congenital abnormality without universal treatment guidelines. An Amplatzer PFO occlusion device can provide a novel method of shunt closure given appropriate shunt type, size and anatomy.

Congenital intrahepatic portosystemic shunt with invasive hepatocellular carcinoma.

A 60-year-old man with a history of congenital intrahepatic portosystemic shunt (CIPS) presented with acute bilateral lower-extremity edema. There were no stigmata of chronic liver disease. However, mildly abnormal liver function tests and α-fetoprotein 79.1 ng/mL were noted. Subsequent imaging revealed a 8 cm right lobe liver mass, extending into the right hepatic vein into the IVC and right atrium (A,B). An 8 mm side-to-side intrahepatic shunt between the right portal vein and the IVC (C,D – CT; E - US) was confirmed.

Congenital intrahepatic portosystemic shunt presenting with muco-cutaneous bleeding

The Journal is the primary organ of Continuing Paediatric Medical Education in Sri Lanka. The journal also has a website. Free full text access is available for all readers.The Sri Lanka Journal of Child Health is now indexed in SciVerse Scopus (Source Record ID 19900193609), Index Medicus for South-East Asia Region (IMSEAR), CABI (Centre for Agriculture and Bioscience International Global Health Database), DOAJ and is available in Google, as well as Google Scholar.The policies of the journal are modelled on the Committee on Publication Ethics (COPE) Guidelines on Principles of Transparency and Best Practice in Scholarly Publishing. Sri Lanka Journal of Child Health is recognised by the International Committee of Medical Journal Editors (ICMJE) as a publication following the ICMJE Recommendations.