Polysplenia with agenesis of the dorsal pancreas and preduodenal portal vein, about a case

Elhattabi, Khalid; Elyamine, Othmane; Bouali, Mounir; Bakouri, Abdelilah El; Bensardi, Fatimazahra; Fadil, Abdelaziz

doi:10.1016/j.ijscr.2020.10.005

Cited by 3 publications

(11 citation statements)

References 17 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…They are about of equal size, associated with the main spleen in the left hypochondrium and vascularized by an artery with normal birth. Polysplenia must be differentiated from supernumerary spleens (which generally measure only 1–2 cm) and from splenosis [ 3 ]. Congenital preduodenal portal vein (PDPV), often present (75%) in LI, is characterized by a portal vein passing in front of the duodenum [ 3 , 7 – 11 ].…”

Section: Discussionmentioning

confidence: 99%

“…Polysplenia must be differentiated from supernumerary spleens (which generally measure only 1–2 cm) and from splenosis [ 3 ]. Congenital preduodenal portal vein (PDPV), often present (75%) in LI, is characterized by a portal vein passing in front of the duodenum [ 3 , 7 – 11 ]. PDPV is rare as an isolated variation (25%) [ 4 , 11 ].…”

Section: Discussionmentioning

confidence: 99%

“…Duodenal obstruction caused by PDPV alone is a very rare situation with only a few reported cases. The reason is probably the fact that PDPV is a thin-walled vessel under low pressure [ 3 , 11 ]. As reported in our case, patients with PDVP may reach adulthood without any symptoms, the anomaly being often found during examinations or surgery for unrelated conditions.…”

Section: Discussionmentioning

confidence: 99%

“…Such accidents can be prevented by performing previous careful diagnostic imaging and especially by searching and noting the possibility of PDPV in cases of polysplenia syndrome. In 25% of cases, but not in this case, a median liver is present in LI (but also in RI) with two symmetrical hepatic lobes and a gallbladder interposed between them [ 3 ]. Variations of the digestive tract associated with LI may comprise an intraperitoneal annular pancreas or sometimes, as illustrated in our case, a short pancreas with agenesis of the corpus and tail [ 3 , 8 ].…”

Section: Discussionmentioning

confidence: 99%

“…The ventral bud gives rise to the head and uncinate process, while the dorsal bud gives rise to the body and tail. The development of pancreatic dorsal bud and spleen occurs in the dorsal mesogastrium [ 3 , 8 , 9 ]. As a consequence, simultaneous anomalies in both these organs can be expected in LI (polysplenia).…”

Section: Discussionmentioning

confidence: 99%

See 4 more Smart Citations

Asymptomatic left isomerism with preduodenal portal vein: computed tomography appearance

Coulier

2021

Surg Radiol Anat

View full text Add to dashboard Cite

Left isomerism (polysplenia), one of the two major variants of heterotaxia with right isomerism (asplenia), may be rarely diagnosed in adulthood. Most cases are nevertheless asymptomatic and incidentally detected during imaging or surgery performed for unrelated conditions. We hereby report a case of left isomerism fortuitously diagnosed in a 55-year-old man with unrelated tachy-cardiomyopathy. Thoraco-abdominal computed tomography revealed a typical preduodenal portal vein (PDPV) associated with a large series of other occult anatomic variations comprising: polysplenia, agenesis of both pancreatic body and tail, complete non-rotation of the bowel and finally azygous continuation of the inferior vena cava. Subtle but highly specific thoracic features of left isomerism were also found with a bilobed right lung and bilateral long hyparterial main bronchi. The features of adult left isomerism are remembered with special attention to the PDPV.

show abstract

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

See 3 more Smart Citations

Asymptomatic left isomerism with preduodenal portal vein: computed tomography appearance

Coulier

2021

Surg Radiol Anat

View full text Add to dashboard Cite

show abstract

Association of Agenesis of the Dorsal Pancreas With HNF1B Heterozygote Mutation: A Case Report

Guo

et al. 2021

Front. Endocrinol.

View full text Add to dashboard Cite

BackgroundAgenesis of the dorsal pancreas (ADP) is a rare disease, the pathogenic mechanism of which is partially related to variants of hepatocyte nuclear factor 1B (HNF1B) gene.Case PresentationWe report a case of ADP, which presented with acute ketoacidosis, hyperuricemia, and liver dysfunction. In this case, the HNF1B score was estimated as 16 and a heterozygous variant of HNF1B in exon 2 (c.513G>A-p.W171X) was identified through gene sequencing.ConclusionsA good understanding of the clinical comorbidities of ADP is essential for avoiding missed diagnosis to a great extent. Moreover, estimation of HNF1B score is recommended before genetic testing.

show abstract

Agenesis of dorsal pancreas as incidental finding in a COVID-19 85-year-old patient: A case report with a review of literature

Branković,

Gmizić,

Dukić

et al. 2024

Medicinska istraživanja

View full text Add to dashboard Cite

Introduction: Polysplenia syndrome is a very rare congenital condition with multifactorial inheritance. It is characterized by the malposition of the thoracic and abdominal organs with or without vascular abnormalities. It comprises multiple cardiac, gastrointestinal, hepatosplenic, pancreatic and renal disorders. It is usually an incidental finding on an abdominal ultrasound or computed tomography (CT) scan performed for other reasons. Patient review: In this case, an 85-year-old male patient is presented, who tested positive for coronavirus disease 2019 (COVID-19) infection, and he was admitted to hospital for bilateral COVID-19 pneumonia. CT scan was performed and revealed polysplenia, various vascular abnormalities, gastrointestinal malformations and agenesis of the dorsal pancreas, so heterotaxy syndrome was diagnosed. Interestingly, this patient did not have diabetes mellitus in spite of agenesis of the dorsal pancreas, neither had he ever have pancreatitis. Conclusion: This present case shows that the quality of life in patients with polysplenia syndrome does not have to be seriously impaired and that these patients can experience old age. Moreover, the awareness and prior knowledge of anomalies included in this syndrome are crucial in order to avoid complications during surgical procedures and/or interventions.

show abstract

Polysplenia with agenesis of the dorsal pancreas and preduodenal portal vein, about a case

Cited by 3 publications

References 17 publications

Asymptomatic left isomerism with preduodenal portal vein: computed tomography appearance

Asymptomatic left isomerism with preduodenal portal vein: computed tomography appearance

Association of Agenesis of the Dorsal Pancreas With HNF1B Heterozygote Mutation: A Case Report

Agenesis of dorsal pancreas as incidental finding in a COVID-19 85-year-old patient: A case report with a review of literature

Contact Info

Product

Resources

About