Background/aim: Anatomic vascular abnormalities of the hepatic arteries are frequent. The aim of the study was to analyze the influence of hepatic arterial variations on postoperative morbidity and resection margin status after pancreatoduodenectomy (PD).
Materials/methods: Patients who underwent PD over a 7-year period (2010-2017) were included in the study. Patients with variant hepatic arterial anatomy were matched 1:2 for age, sex, ASA score, and histology.
Results: A total of 232 patients underwent PD. Variant hepatic arterial anatomy was found in 35 (15.1% of the total patient population). The most common variation was an accessory right hepatic artery (8.19%) and a replaced right hepatic artery (5.60%) arising from the superior mesenteric artery. These 35 patients were compared with 70 patients with no hepatic artery variations. Postoperative surgical complications occurred in 12.1% and 26.5% (P = 0.08) and in-hospital mortality was 6% and 5.4% ( P = 0.99) between patients with and without variant hepatic arteries. There was no difference in positive resection margins (R1) (18.2% vs 20.5%, P = 0.99) between the two groups.
Conclusions: An aberrant hepatic artery does not increase morbidity or R1 resection in patients undergoing PD.
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http://dx.doi.org/10.1002/jso.25461 | DOI Listing |
J Med Case Rep
January 2025
Department of Hepatic Biliary Pancreatic Medicine, First Hospital of Jilin University, 1 Xinmin Avenue, Changchun, 130021, China.
Background: Dyskeratosis congenita is a rare genetic disease due to telomere biology disorder and characterized by heterogeneous clinical manifestations and severe complications. "Porto-sinusoidal vascular disease" has been recently proposed, according to new diagnostic criteria, to replace the term "idiopathic non-cirrhotic portal hypertension." TERT plays an important role in telomeric DNA repair and replication.
View Article and Find Full Text PDFJ Neural Transm (Vienna)
January 2025
Section of Adult Neurology, Department of Internal Medicine, Chong Hua Hospital, Fuente, Cebu, Philippines.
Joubert Syndrome (JS) is a congenital cerebellar ataxia typically inherited in an autosomal recessive pattern, although rare X-linked inheritance can occur. It is characterized by hypotonia evolving into ataxia, global developmental delay, oculomotor apraxia, breathing dysregulation, and multiorgan involvement. To date, there are 40 causative genes implicated in JS, all of which encode proteins of the primary cilium.
View Article and Find Full Text PDFEur J Med Genet
January 2025
APHM, Timone Enfant, Service de pédiatrie multidisciplinaire, Marseille France; Aix Marseille Univ, INSERM, MMG, Marseille France.
Pathogenic variants in VPS53 are associated with pontocerebellar hypoplasia type 2E (PCH2E), characterized by microcephaly, severe neurodevelopmental impairment and epilepsy. We present a case of a female neonate with VPS53 pathogenic variants exhibiting the classic phenotypic features along with liver disease and deafness, which had not been described in previously reported cases. Similarly, while liver abnormalities have been reported in patients with mutations in other genes coding for proteins of the GARP or EARP complex, of which VPS53 is a subunit, liver disease has not been described in PCH2E until now.
View Article and Find Full Text PDFArq Bras Cir Dig
January 2025
Mongi Slim Hospital, Department of Pathology - Marsa, Tuni, Tunísia.
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