Transhepatic ultrasound guided embolization as a successful novel technique in treatment of pediatric complex intrahepatic arterioportal fistula: a case report and review of the literature

(2023) 17:412 Taher et al. Journal of Medical Case Reports https://doi.org/10.1186/s13256-023-04047-0 Journal of Medical Case Reports Open Access CASE REPORT Transhepatic ultrasound guided embolization as a successful novel technique in treatment of pediatric complex intrahepatic arterioportal fistula: a case report and review of the literature Heba Taher1*, ElSayed Kidr1, Ahmed Kamal1, Mohamed ElGobashy2, Shady Mashhour2, Amr Nassef2, Sherifa Tawfik3, Gamal El Tagy2, Muayad Shaban1, Haytham Eltantawi1 and Khaled S. Abdullateef1 Abstract Introduction Intrahepatic vascular shunts “IHVS” are abnormal communications between intra-hepatic vasculature involving the arterial, portal, or hepatic venous system. Arterio-portal fistula “APF” is an intrahepatic communication between the hepatic arterial system and the portal venous system without any communication with the systemic venous circulation. APF is considered a rare cause of portal hypertension and gastrointestinal bleeding in infancy. Case presentation A 3-month-old Mediterranean female with known cardiac congenital anomalies presented to us with abdominal distension and diarrhea. Ultrasonography revealed massive ascites and computerized tomography (CT) abdomen with intravenous (IV) contrast revealed a left hepatic lesion. On further evaluation, an intrahepatic arterio-portal vascular malformation was detected. Attempted trans arterial embolization failed and radiology team successfully carried out direct trans hepatic ultrasound guided coiling of the aneurysmal venous sac followed by successful resection of segment 4 of the liver with the vascular malformation avoiding life threatening intra operative bleeding. Conclusion Any child with recurrent gastrointestinal bleeding, failure to thrive, vomiting, diarrhea, steatorrhea, splenomegaly, or ascites should be investigated for intrahepatic arterio-portal fistula “IAPF”. Our novel technique of direct trans hepatic ultrasound guided coiling is an alternative method if trans arterial embolization “TAE” failed. Keywords Intrahepatic vascular shunts, Portal hypertension, Congenital arterioportal fistula, Trans hepatic embolization, Transarterial embolization *Correspondence: Heba Taher 1 Pediatric Surgery Department, Specialized Pediatric Hospital, Cairo University Kasr Al Einy, Faculty of Medicine, 1 Abou El Rish Sq., El Sayeda Zeinab, Cairo, Egypt 2 Department of Radiology, Cairo University Kasr Al Einy, Faculty of Medicine, Cairo, Egypt 3 Pathology Department, Ministry of Health, Cairo, Egypt Introduction Arterio-portal fistula “APF” is an intrahepatic communication between the hepatic arterial system and the portal venous system without any communication with the systemic venous circulation. The etiology of APF may be congenital “Primary” or acquired “Secondary”. The acquired form is most often due to trauma [1], surgical procedures [2–4] or rupture hepatic artery aneurism © The Author(s) 2023. Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativeco mmons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data. Taher et al. Journal of Medical Case Reports (2023) 17:412 [5], transhepatic intervention [6] or biopsy [7, 8]. On the other hand, congenital arterioportal fistulae are rare, with only a small number of case reports describing this entity in children—44 cases—we report here the 45th case of a congenital APF which is a complex type of IHAPF and treated with a Novel technique due to failure of intrahepatic arterial embolization. Surgical treatment was not considered the first option because of the aberrant vasculature and the major concern about uncontrolled intraoperative bleeding. This case is unique because it highlights the importance of preoperative radiological interventions preoperative to reduce risks of fatal intraoperative bleeding, even in failed attempts of radiological interventions there are novel techniques to achieve ideal preoperative control. Case presentation A 3-month-old Mediterranean female, full term after cesarean section “CS”, Apgar score of 8 which was normal and normal birth weight. with cardiac anomalies in the form of atrial septal defect “ASD”—3 × 3.5 ml—and patent foramen ovale “PDA” -closed spontaneously-, presented with failure to thrive, abdominal distention and diarrhea. Physical examination showed hepatomegaly Page 2 of 8 and superficial dilated veins which are suggestive of portal hypertension. Ultrasound revealed massive ascites. On at admission the child weight was 2.5 kg and length 49 cm both were on the low centile for age. There was negative family history of medical diseases and negative consanguinity hemoglobin was 8.6 g/dl, albumin was 2.8 g/dl, and prealbumin 13.6 mg/dl. Laboratory values were white blood cell count 10.6 × 103/ mm3, platelet count 500 × 103/mm3, normal prothrombin and partial thromboplastin times, serum sodium 120 mmol/l, potassium 6.2 mmol/l, chloride 80 mmol/l, CO2 22 mmol/l, urea 26 mg/dl, creatinine 0.5 mg/dl, aspartate aminotransferase 105 U/l, alanine aminotransferase 65 U/l, alkaline phosphatase 248 U/l, γ-glutamyl transferase 167 U/l, total bilirubin 0.8 mg/dl. CT abdomen with IV contrast was done and revealed a well-defined lesion at the left lobe of the liver, which was suspected to be hemangioendothelioma, all liver and kidney functions were normal. MSCT angiography and portography “Multi-slice computed tomography” was done and revealed complex intrahepatic arterio-portal vascular malformation (Figs. 1, 2, 3) in segment 4 between an apparent atypical branch of the hepatic artery proper and the left portal vein, the apparent branch was communicating with a dilated venous cystic structure which was Fig. 1 Multislice CT hepatic angiography and portography; a soft tissue enhancing mass (Green arrow), b intensely enhancing cystic lesion (vascular space) enhancing during the arterial phase vascular arterial lesion (red arrow) Taher et al. Journal of Medical Case Reports (2023) 17:412 Fig. 2 Portal phase showing early opacification of portal vein. The a (...truncated)