Analysis of associated malformations by computed tomography in adults with polysplenia syndrome: A pilot study

Xinru Gu; Shuangshuang Xu; Jinghua Chen; Xiaoqin Jiang; Ping Xie; Xiang Fang; Yan Gao; Jian Huang; Kefu Liu

doi:10.1371/journal.pone.0312548

. 2024 Dec 20;19(12):e0312548. doi: 10.1371/journal.pone.0312548

Analysis of associated malformations by computed tomography in adults with polysplenia syndrome: A pilot study

Xinru Gu ^1,^#, Shuangshuang Xu ^2,^#, Jinghua Chen ^3,^#, Xiaoqin Jiang ^1,^#, Ping Xie ^1,^#, Xiang Fang ⁴, Yan Gao ⁵, Jian Huang ³, Kefu Liu ^1,^*

Editor: Gurinder Kumar⁶

PMCID: PMC11661641 PMID: 39705258

Abstract

Objective

To analytically depict the associated malformations of polysplenia syndrome (PS) in adults via computed tomography (CT).

Materials and methods

The incidence of malformations associated with PS in twelve adult patients was retrospectively analyzed via CT imaging.

Results

The number of splenic nodules ranged from three to twelve; the splenic nodules were located in the left upper quadrant in nine patients and in the right upper quadrant in three patients. A short pancreas was present in all twelve patients. Midgut malrotation was present in eight patients. Situs inversus totalis was present in two patients. Nine patients presented the absence of hepatic segmental inferior vena cava (IVC), with the hepatic vein directly converging into the right atrium and the continuation of the azygos vein. The preduodenal portal vein was present in six patients. Left lung heterotaxy was found in nine patients. The inferior vena cava was bilateral in one patient. Aberrant right subclavian arteries, bilateral common carotid arteries sharing trunks, abnormal renal vein branching and routing, and abdominal portal vein branching were also found in individual patients.

Conclusions

PS is a complex malformation syndrome involving multiple systems. The most common malformation is short pancreas, and other malformations, such as left lung heterogeneity, hepatic segmental IVC agenesis with continuation of the azygos vein, midgut malrotation, preduodenal portal vein, and left atrial heterotaxy, have relatively high prevalence rates.

Introduction

Polysplenia syndrome (PS), a rare syndrome of congenital anomalous spleen development with other multisystem malformations [1], is also known as left atrial isomerism syndrome (LAIS) because of the frequent occurrence of left atrial isomerism [2]. PS presenting in adults is very rare and has been reported mostly in case studies [3–5]. Compared with other examinations, thoracic and abdominal multilayer computed tomography (CT) is the best means to clarify the details of PS combined with various anatomical variants [6]; therefore, in this study, we retrospectively analyzed the CT imaging data of twelve adults with PS to summarize the concomitant deformities in adults with PS with the aim of improving the understanding of concomitant deformities of PS, avoiding misdiagnosis of anatomical variants as lesions and preventing unnecessary surgical injuries.

Materials and methods

Patients

This retrospective study was approved by the institutional review board (No. 2023177, The Affiliated Suzhou Hospital of Nanjing Medical University) with a waiver of written informed consent.

CT images of 12 PS patients from January 2015 to March 2024 were retrospectively collected. The data for this study were accessed from 10/10/2023 to10/10/2024. There were 5 men and 7 women, with an age range of 30–74 years. All 12 patients underwent plain CT examination of the chest and abdomen, and CT enhancement was performed for 6 patients.

Analysis methods

Two radiologists with 7 and 20 years of experience in diagnostic imaging observed and analyzed the CT images of each patient with PS, respectively, and the concomitant deformities of the PS were recorded.

Results

Medical history

A history of cholangiocarcinoma with cirrhosis, cholangiocarcinoma in the porta hepatis with cholecystitis, chronic inflammation of the bile ducts and duodenum, biliary stones, arrhythmia and right kidney stones, left kidney stones, polyps of the colon, and ventricular septal defect (VSD) were recorded in one patient each, and the other patients did not have any special medical history (Table 1).

Table 1. Clinical data of 12 patients with polysplenic syndrome.

Case No.	Age (Year)	Sex	Medical history
1	70	F	Polyp of the colon
2	38	M	VSD
3	53	F	None
4	38	F	None
5	74	M	Cholangiocarcinoma, cirrhosis
6	69	F	Inflammation of the bile ducts and duodenum
7	64	F	Gallstones
8	66	M	Cholangiocarcinoma, Cholecystitis
9	31	M	Arrhythmia, right kidney stone
10	30	M	None
11	57	F	Left kidney stone
12	65	F	None

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VSD- ventricular septal defect

CT imaging findings

The concomitant deformities of the PS of 12 patients shown on CT are summarized in Table 2.

Table 2. The malformations of 12 patients with polysplenic syndrome on CT.

Malformations	Percentage of Occurrence	Case1	Case2	Case3	Case4	Case5	Case6	Case7	Case8	Case9	Case10	Case11	Case 12
Left lung heterogeneity	75%	Yes	Yes	Yes	Yes	Yes	No	Yes	Yes	No	No	Yes	Yes
Left atrial heterotaxy	42%	Yes	Yes	No	No	Yes	No	Yes	Yes	No	No	No	No
Location of spleen		Left	Left	Right	Left	Left	Left	Left	Left	Left	Left	Right	Right
Number of spleens		5	12	4	7	4	3	11	5	12	5	8	5
Liver: small left lobe	8%	Yes	No	No	No	No	No	No	No	No	No	No	No
Left liver	17%	No	No	Yes	No	No	No	No	No	No	No	No	Yes
Horizontal liver		No	No	No	No	No	No	Yes	Yes	No	No	Yes	No
Location of gallbladder		Right	Right	Left	Right	Right	Right	Right	Median	Right	Right	Right	Left
Short pancreas	100%	Yes	Yes	Yes	Yes	Yes	Yes	Yes	Yes	Yes	Yes	Yes	Yes
Midgut malrotation	67%	Yes	Yes	Yes	No	Yes	No	Yes	Yes	Yes	No	Yes	No
Hepatic segmental IVC agenesis with continuation of AZ	75%	Yes	No	No	Yes	Yes	No	Yes	Yes	Yes	Yes	Yes	Yes
Bilateral IVC	8%	No	No	Yes	No	No	No	No	No	No	No	No	No
PDPV	42%	No	Yes	No	Yes	Yes	No	No	No	Yes	Yes	Yes	No
Double LRV	8%	Yes	No	No	No	No	No	No	No	No	No	No	No
Double RRV	8%	No	No	Yes	No	No	No	No	No	No	No	No	No
Bilateral RV inflow AZ	8%	No	No	No	No	No	No	No	Yes	No	No	No	No
LRV travels behind AA, and bilateral RV flow into AZ	8%	No	No	No	No	No	No	No	No	Yes	No	No	No
RRV flows into AZ, LRV flows into HAZ	17%	No	No	No	No	No	No	No	No	No	No	Yes	Yes
Bilateral CCA shared trunks	17%	Yes	No	No	No	No	No	No	Yes	No	No	No	No
ARSA	17%	Yes	No	No	No	No	No	No	Yes	No	No	No	No
RAA	17%	No	No	Yes	No	No	No	No	Yes	No	No	No	No
DPT	17%	No	No	Yes	No	No	No	No	Yes	No	No	No	No
Dextrocardia	17%	No	No	Yes	No	No	No	No	Yes	No	No	No	No
SIT	17%	No	No	Yes	No	No	No	No	No	No	No	No	Yes
The anterior branch of the portal vein enters the liver from the anterior edge of the liver	8%	Yes	No	No	No	No	No	No	No	No	No	No	No

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IVC-inferior vena cava; AZ-azygos vein; PDPV-preduodenal portal vein; RV-renal vein; LRV-left renal vein; RRV- right renal vein; AA-abdominal aorta; SVC-superior vena cava; HAZ-hemiazygos vein; ARSA- aberrant right subclavian artery; CCA-common carotid artery; SIT-Situs inversus totalis; RAA-right aortic arch; DPT-dextral pulmonary trunk

Respiratory system: Nine patients (75%, 9/12) presented with left lung heterogeneity (i.e., both the lungs and bilateral bronchi presented with left-sided morphology) (Fig 1a).

Spleen: The number of splenic nodules ranged from three to twelve, with sizes ranging from 3×4×6 mm to 18×30×88 mm (Fig 1b). Polysplenia nodules were located in the left upper quadrant in nine patients (75%, 9/12) and in the right upper quadrant (25%, 3/12) in three patients (Fig 1b). Splenic nodules were distributed along the external and posterior parts of the greater curvature of the stomach (Fig 1b).

Pancreas: A short pancreas was observed in all twelve patients (100%, 12/12) (the head and uncinate process of the pancreas were enlarged, and the body and tail of the pancreas were short or absent) (Fig 1c).

Hepatobiliary system: Six patients exhibited normal anatomy (50%, 6/12). The liver and gallbladder were found in the left upper quadrant in two patients (17%, 2/12). A small left lobe of the liver was detected in one patient (8%, 1/12), a horizontal liver with a right gallbladder was detected in two patients (17%, 2/12) (Fig 1d), and a horizontal liver and median gallbladder were detected in one patient (8%, 1/12) (Fig 1e).

Gastrointestinal tract: The stomach was located on the right side (Fig 1c) in three patients (25%, 3/12), and midgut malrotation was found in eight patients (67%, 8/12) (Fig 1f).

Heart: Dextrocardia was found in two patients (17%, 2/12) (Fig 1g).

Atrium: CT suggested possible left atrial heterotaxy in five patients (42%, 5/12) (Fig 1h).

Aortic arch: A right aortic arch (RAA) and dextral pulmonary trunk were observed in two patients (17%, 2/12) (Fig 1i).

Superior vena cava (SVC): A persistent left superior vena cava (PLSVC) was found in one patient (8%, 1/12).

Inferior vena cava (IVC): The absence of the hepatic segment of the IVC (Fig 1j and 1k), which continued upward as a thickened azygos vein was found in nine patients (75%, 9/12) (Fig 1k), and a bilateral IVC was found in one patient (8%, 1/12) (Fig 1f).

Portal vein: The preduodenal portal vein (PDPV) was present in six patients (50%, 6/12) (Fig 1l). In one patient (8%, 1/12), the portal vein traveled in front of the head of the pancreas and then bifurcated into two branches; the thicker branch traveled along the anterior border of the liver to the diaphragmatic surface of the liver into the liver, and the thinner branch entered the liver in the first hepatic portal (Fig 1m).

Hepatic vein: In nine patients (75%, 9/12), the hepatic vein exited the second porta hepatis and converged directly into the right atrium (Fig 1k).

Renal veins: Two patients (17%, 2/12) had unilateral renal veins with double-branched variants: one patient had both renal veins converging directly into the azygos vein (Fig 1n), one patient had the left renal vein traveling posterior to the abdominal aorta (Fig 1o), and one patient had the right renal vein flowing back into the azygos vein and injecting upward into the SVC. In two patients (17%, 2/12), the left renal vein first converged into the hemiazygos vein and then into the azygos vein in the posterior mediastinum.

Common carotid artery (CCA): Bilateral CCAs shared trunks in two patients (17%, 2/12) (Fig 1p).

Others: An aberrant right subclavian artery (ARSA) was found in two patients (17%, 2/12) (Fig 1p).

Discussion

Pathogenesis and survival rates

PS was first reported by Baillie in 1788 [7], but the exact cause of PS is still unclear, and studies have shown that its occurrence is closely related to embryonic development, inheritance, teratogenic factors, genetic mutations and other factors [6]. The 5th–7th week of embryo development is a critical stage for the development and rotation of the spleen, atrial septum, trunk of the conus artery and atrioventricular valve, as well as the process of the gastrointestinal tract returning to the abdominal cavity from the umbilical cord for rotation. Cardiovascular and visceral anomalies occur when this process is impaired. Situs solitus describes the normal anatomic position of organs or structures. Situs inversus refers to a left–right inversion, resulting in the mirror image of situs solitus. Situs ambiguous, a synonym for left or right isomerism, characterizes the positions of organs or structures lateralizing across the left–right axis and cannot be categorized as situs solitus or inversus. Any type of situs other than situs solitus can be described as heterotaxy [8].

PS is very rare, with an infantile morbidity rate of only 1/250000, and the degree of congenital heart disease malformation affects the survival of patients with PS. The mortality rate in the first year is 50–60%; 25% of patients with PS survive to the age of 5 years, and only 10% have normal hearts or only minor heart defects and therefore survive to adulthood [9–11]. Some studies have reported [4, 9] that the prevalence of PS is greater in females than in males, and our findings are consistent with the literature.

CT findings

Multiple spleens

The number of spleens reported in the literature is between two and sixteen [10], with different sizes, no main spleen, no increase in the total weight of the spleen, and no hypersplenism, and the spleen can be located in the left upper quadrant, right upper quadrant, or both sides of the abdomen, with the left upper quadrant being the most common. The spleen is usually located on the side of the greater curvature of the stomach and shows "Zebra pattern" enhancement in the arterial phase and uniform enhancement in the venous and delayed phases. The degree of enhancement is consistent with that of a normal spleen [6, 11].

The number of spleens in this group ranged from three to twelve, twelve of which were located next to the greater curvature of the stomach and, three of which were located in the right upper quadrant. Six patients who completed the CT enhancement examination had an arterial "Zebra pattern" and uniform venous enhancement, twelve patients had no main spleen, and the size of the splenic nodules varied, which was consistent with previous reports.

Visceral abnormalities

In our group, all twelve patients had short pancreas, including two patients with pancreatic inversion, which was the most prevalent extrasplenic abnormality. Some studies [12–15] have reported that pancreatic abnormalities are associated with PS. The pancreas is formed by the fusion of ventral and dorsal pancreatic buds; the ventral pancreatic buds give rise to the uncinate process and head, and the dorsal pancreatic buds give rise to the body and tail [14]. When the pancreas is dorsally underdeveloped, dysplasia of the body and tail of the pancreas, occurs which mostly manifests as a short pancreas [14].

In this group, one patient had a small left lobe of the liver accompanied by a right gallbladder, two patients had total visceral inversion manifesting as a left liver and gallbladder, two patients had a horizontal liver with a right gallbladder, and one patient had a horizontal liver with a median gallbladder. Some previous studies have also reported these hepatobiliary anomalies [4, 12].

In our group, there were eight cases of midgut malrotation (8/12, 67%), including two cases of gastrointestinal anteversion (total visceral anteversion), which is consistent with the published literature showing small bowel malrotation in 60.4% of PS patients [4, 16].

In our group, we found kidney stones in two of our twelve patients, and no other genitourinary anomalies, such as a double ureter or renal hypoplasia [17, 18], were observed.

Vascular malformations

In our group, hepatic segmental IVC agenesis with continuation of azygos was found in nine patients. When the hepatic segment of the IVC fails to anastomose with the suprarenal segment during embryonic development, the renal segment and lower segment flow back into the SVC via the azygos or hemiazygos vein [6], which is considered the most common vascular malformation in PS [9, 19, 20].

In our group, PDPV was present in six patients. PDPV [21, 22] is a rare congenital anomaly, especially in adults, in which the portal vein passes through the anterior part of the duodenum rather than the posterior part and leaves an abnormal vein in the anterior part of the duodenum due to an anomaly of rotation during intestinal rotation or an anomaly of closure of the vitelline vein (umbilical vein), which results in the formation of a portal vein. In patients who require surgery, failure to detect the presence of PDPV before surgery may lead to serious complications such as intraoperative bleeding. In addition, it has been reported [23] that PDPV may compress the duodenum and biliary tract, causing intestinal obstruction and biliary obstruction.

Renal vein anomalies mainly manifest as the retro-aortic renal vein and the right renal vein flowing back into the azygos vein to inject upward into the SVC, and the left renal vein converges into the hemiazygos vein, which converges into the azygos vein in the posterior mediastinum [4]. Among them, the retro-aortic renal vein usually occurs unilaterally on the left side, which can lead to hematuria, left-sided pain, and a variety of other symptoms, often suggesting posterior nutcracker syndrome [24], which is relatively rare and is consistent with the presentation in one patient in our group.

Other vascular malformations, including ARSA and bilateral CCA shared trunks [4], were found in our group, with the exception of vertebral artery origin anomalies. In our study, two special manifestations were observed that were not reported in the previous PS-related literatures: bilateral IVC and portal vein abnormalities bifurcated into two branches with the thicker branch traveling along the anterior border of the liver to the diaphragmatic surface of the liver to enter the liver and the thinner branch entering the liver at the first hepatic hilar.

Lung anomalies

Nine patients in our group exhibited left lung heterogeneity, with a higher incidence than that reported in previous studies, which reported an incidence of 55% [10]. A previous study [6] also revealed that the incidence of bilateral lung heterogeneity on the left side was much lower in adults with PS, which suggested that since adults with PS usually do not have cardiac anomalies such as abnormal pulmonary venous return or atrial septal defect (ASD), they do not experience bilateral left-sided changes. In our group, there were only two patients with cardiac abnormalities, which is not consistent with previous reports in the literature [6, 10]. The reason for this may be that left lung heterogeneity is related not only to cardiac abnormalities but also to other factors that need further confirmation.

Cardiac abnormalities

In this group, five CT scans suggested the possibility of left atrial heterotaxy, one had a history of VSD, and cardiac ultrasound suggested a tumor in the membranous portion of the interventricular septum. PS in adults is usually not associated with cardiac developmental abnormalities or only with mild cardiac abnormalities [3]. These abnormalities mainly include positional abnormalities of the heart, such as dextrocardia [9]; structural abnormalities, such as ASD, VSD, right ventricular double outlet, atrial septal hypertrophy, and lipoma [11]; and arrhythmias, such as atrioventricular block and bradycardia [25], some of which are difficult to diagnose via CT and usually rely on cardiac ultrasound and electrocardiogram.

Comorbid tumors

In our group, there were two patients with cholangiocellular carcinoma, which was also reported previously [26], and the cause is hypothesized to be related to biliary obstruction due to developmental malformations of the biliopancreatic duct and recurrent infections of the biliary system in patients with PS. Previous case reports [15, 26–28] have shown that PS can co-occur with malignant tumors, such as hepatocellular carcinoma, intrahepatic cholangiocarcinoma, gastric carcinoma, rectal carcinoma, and breast cancer. However, there is no evidence that PS is a precancerous syndrome or that there is a greater incidence of tumors than in the normal population.

Differential diagnosis

PS needs to be differentiated from asplenia syndrome (AS), accessory spleen, simple visceral inversion, enlarged lymph nodes in the abdominal cavity, and other nodular masses in the abdominal cavity [7, 9, 29]. (1) AS can present as congenital splenic hypoplasia or absence of the spleen, with trilobarism of both lungs (right-sided heterotaxy of both lungs), a median liver, and more frequent and more severe cardiovascular malformation than PS, and the IVC is located on the same side of the spine as the aorta. (2) Accessory spleen: There is a large primary spleen, with a large difference in volume between the primary and secondary spleens, whereas there is no primary spleen in PS. (3) Simple inversion of the viscera: partial or total inversion of the viscera, resembling the mirror image of a normal person, is not associated with cardiovascular or visceral developmental malformations. (4) Enlarged lymph nodes in the abdominal cavity and other nodular masses in the abdominal cavity: There is a history of inflammation or malignant tumors, often multiple, located in the abdominal cavity and retroperitoneum, without a splenic hilar incision or splenic hilar vessels, and homogeneous enhancement in all three phases is observed.

Conclusions

PS is a complex malformation syndrome involving multiple systems. The most common malformation is a short pancreas, and other malformations, such as left lung heterogeneity, hepatic segmental IVC agenesis with continuation of the azygos vein, midgut malrotation, preduodenal portal vein, and left atrial heterotaxy, have relatively high prevalence.

Being familiar with the occurrence of malformations associated with PS is conducive to avoiding misdiagnosis and mistreatment. Because the malformations involve multiple systems, multidisciplinary consultation is more conducive to the evaluation of this disease.

Data Availability

All relevant data are within the manuscript.

Funding Statement

The funding for the research has been provided by " Suzhou key diseases project (LCZX202212), Suzhou Science and Technology Bureau Project (SKY2023064, SKYD2023255)" and the funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.

References

1.Abdullah RA, Alharbi MA, Alnutaifi FM, Faqih AI, Alduraibi KM, Alharbi AS. Polysplenia Syndrome Complicated by Splenic Infarction: A Report of a Rare Case. Cureus. 2023;15(5):e38589. Epub 2023/06/07. doi: 10.7759/cureus.38589 . [DOI] [PMC free article] [PubMed] [Google Scholar]
2.Frescura C, Ho SY, Giordano M, Thiene G. Isomerism of the atrial appendages: morphology and terminology. Cardiovasc Pathol. 2020;47:107205. Epub 20200116. doi: 10.1016/j.carpath.2020.107205 . [DOI] [PubMed] [Google Scholar]
3.Malki MC, Outznit M, Mechhor S, Bouibaouen B, Nkurunziza L, Bacha HE, et al. Polysplenia syndrome in adulthood: a case report. Pan Afr Med J. 2022;41:67. Epub 2022/04/05. doi: 10.11604/pamj.2022.41.67.29014 . [DOI] [PMC free article] [PubMed] [Google Scholar]
4.Rameshbabu CS, Gupta KK, Qasim M, Gupta OP. Heterotaxy Polysplenia Syndrome In An Adult With Unique Vascular Anomalies: Case Report With Review Of Literature. J Radiol Case Rep. 2015;9(7):22–37. Epub 2015/12/03. doi: 10.3941/jrcr.v9i7.2210 . [DOI] [PMC free article] [PubMed] [Google Scholar]
5.Mountassir ME, Borahma M, Benelbarhdadi I, Lagdali N, Hosni A, Ajana FZ. Polysplenia syndrome revealed in adulthood by pancreatic and vascular malformations: a case report. Pan Afr Med J. 2022;43:77. Epub 2023/01/03. doi: 10.11604/pamj.2022.43.77.31496 . [DOI] [PMC free article] [PubMed] [Google Scholar]
6.Tawfik AM, Batouty NM, Zaky MM, Eladalany MA, Elmokadem AH. Polysplenia syndrome: a review of the relationship with viscero-atrial situs and the spectrum of extra-cardiac anomalies. Surg Radiol Anat. 2013;35(8):647–53. Epub 2013/03/20. doi: 10.1007/s00276-013-1100-x . [DOI] [PubMed] [Google Scholar]
7.Vaughan TJ, Hawkins IF Jr., Elliott LP. Diagnosis of polysplenia syndrome. Radiology. 1971;101(3):511–8. Epub 1971/12/01. doi: 10.1148/101.3.511 . [DOI] [PubMed] [Google Scholar]
8.Wolla CD, Hlavacek AM, Schoepf UJ, Bucher AM, Chowdhury S. Cardiovascular manifestations of heterotaxy and related situs abnormalities assessed with CT angiography. J Cardiovasc Comput Tomogr. 2013;7(6):408–16. Epub 2013/12/18. doi: 10.1016/j.jcct.2013.11.008 . [DOI] [PMC free article] [PubMed] [Google Scholar]
9.Gayer G, Apter S, Jonas T, Amitai M, Zissin R, Sella T, et al. Polysplenia syndrome detected in adulthood: report of eight cases and review of the literature. Abdom Imaging. 1999;24(2):178–84. doi: 10.1007/s002619900471 . [DOI] [PubMed] [Google Scholar]
10.Peoples WM, Moller JH, Edwards JE. Polysplenia: a review of 146 cases. Pediatr Cardiol. 1983;4(2):129–37. doi: 10.1007/BF02076338 . [DOI] [PubMed] [Google Scholar]
11.Low JP, Williams D, Chaganti JR. Polysplenia syndrome with agenesis of the dorsal pancreas and preduodenal portal vein presenting with obstructive jaundice—a case report and literature review. Br J Radiol. 2011;84(1007):e217–20. doi: 10.1259/bjr/27680217 . [DOI] [PMC free article] [PubMed] [Google Scholar]
12.Kayhan A, Lakadamyali H, Oommen J, Oto A. Polysplenia syndrome accompanied with situs inversus totalis and annular pancreas in an elderly patient. Clin Imaging. 2010;34(6):472–5. Epub 2010/11/26. doi: 10.1016/j.clinimag.2009.11.011 . [DOI] [PubMed] [Google Scholar]
13.Kobayashi H, Kawamoto S, Tamaki T, Konishi J, Togashi K. Polysplenia associated with semiannular pancreas. Eur Radiol. 2001;11(9):1639–41. Epub 2001/08/21. doi: 10.1007/s003300000757 . [DOI] [PubMed] [Google Scholar]
14.Maier M, Wiesner W, Mengiardi B. Annular pancreas and agenesis of the dorsal pancreas in a patient with polysplenia syndrome. AJR Am J Roentgenol. 2007;188(2):W150–3. Epub 2007/01/24. doi: 10.2214/AJR.05.1859 . [DOI] [PubMed] [Google Scholar]
15.Ito H, Ohgi S, Kanno T, Ishibashi T. Heterotaxy syndrome with pancreatic malrotation: CT features. Abdom Imaging. 2003;28(6):856–8. doi: 10.1007/s00261-003-0034-x . [DOI] [PubMed] [Google Scholar]
16.Burton EC, Olson M, Rooper L. Defects in laterality with emphasis on heterotaxy syndromes with asplenia and polysplenia: an autopsy case series at a single institution. Pediatr Dev Pathol. 2014;17(4):250–64. Epub 20140415. doi: 10.2350/13-11-1406-OA.1 . [DOI] [PubMed] [Google Scholar]
17.Hadar H, Gadoth N, Herskovitz P, Heifetz M. Short pancreas in polysplenia syndrome. Acta Radiol. 1991;32(4):299–301. Epub 1991/07/01. . [PubMed] [Google Scholar]
18.Herman TE, Siegel MJ. Polysplenia syndrome with congenital short pancreas. AJR Am J Roentgenol. 1991;156(4):799–800. Epub 1991/04/01. doi: 10.2214/ajr.156.4.2003448 . [DOI] [PubMed] [Google Scholar]
19.Fulcher AS, Turner MA. Abdominal manifestations of situs anomalies in adults. Radiographics. 2002;22(6):1439–56. doi: 10.1148/rg.226025016 . [DOI] [PubMed] [Google Scholar]
20.Li ZG, Lin ZC, Mu HY. Polysplenia Syndrome With Splenic and Skeletal Muscle Metastases From Thyroid Carcinoma Evaluated by FDG PET/CT: Case Report and Literature Review: A Care-Compliant Article. Medicine (Baltimore). 2016;95(4):e2532. doi: 10.1097/MD.0000000000002532 . [DOI] [PMC free article] [PubMed] [Google Scholar]
21.G AL, Kagali NA, M S, Prasad BS. Preduodenal portal vein in adult with polysplenia syndrome revisited with a case report. Indian J Surg. 2014;76(2):137–42. Epub 2014/06/04. doi: 10.1007/s12262-013-0812-9 . [DOI] [PMC free article] [PubMed] [Google Scholar]
22.Seo HI, Jeon TY, Sim MS, Kim S. Polysplenia syndrome with preduodenal portal vein detected in adults. World J Gastroenterol. 2008;14(41):6418–20. doi: 10.3748/wjg.14.6418 . [DOI] [PMC free article] [PubMed] [Google Scholar]
23.Salma EH, Najwa A, Mehdi T, Nazik A, Latifa C, Siham EH. Polysplenia Syndrome With Persistent Left Superior Vena Cava: Case Report and Review of the Literature. Glob Pediatr Health. 2022;9:2333794X221127640. Epub 20220927. doi: 10.1177/2333794X221127640 . [DOI] [PMC free article] [PubMed] [Google Scholar]
24.Lai B, Zhong J, Zhong J. Polysplenia syndrome complicated with posterior nutcracker syndrome. Asian J Surg. 2023;46(12):5460–1. Epub 2023/08/05. doi: 10.1016/j.asjsur.2023.07.115 . [DOI] [PubMed] [Google Scholar]
25.Montero-Macias R, Krief D, Villefranque V, Kilani M, Castillo-Tunon JM. Anatomy, embryology, and imaging of situs ambiguous with polysplenia and left IVC. Radiol Case Rep. 2023;18(5):1727–32. Epub 20230227. doi: 10.1016/j.radcr.2023.02.005 . [DOI] [PMC free article] [PubMed] [Google Scholar]
26.Yorita K, Sasaki S, Kawada A, Okazaki M, Yamai H, Uchita K, et al. Hepatoid Adenocarcinoma of the Extrahepatic Bile Duct in a Patient with Polysplenia Syndrome. Intern Med. 2017;56(4):401–7. Epub 2017/02/17. doi: 10.2169/internalmedicine.56.7526 . [DOI] [PMC free article] [PubMed] [Google Scholar]
27.Mimatsu K, Oida T, Kano H, Kawasaki A, Fukino N, Kida K, et al. Preduodenal portal vein, intestinal malrotation, polysplenia, and interruption of the inferior vena cava: a review of anatomical anomalies associated with gastric cancer. Surg Radiol Anat. 2012;34(2):179–86. Epub 20111112. doi: 10.1007/s00276-011-0894-7 . [DOI] [PubMed] [Google Scholar]
28.Iwamura T, Shibata N, Haraguchi Y, Hisashi Y, Nishikawa T, Yamada H, et al. Synchronous double cancer of the stomach and rectum with situs inversus totalis and polysplenia syndrome. J Clin Gastroenterol. 2001;33(2):148–53. Epub 2001/07/27. doi: 10.1097/00004836-200108000-00012 . [DOI] [PubMed] [Google Scholar]
29.Yoneyama H, Kondo C, Yamasaki A, Nakanishi T, Sakai S. Comparison of situs ambiguous patterns between heterotaxy syndromes with polysplenia and asplenia. Eur J Radiol. 2015;84(11):2301–6. Epub 2015/08/26. doi: 10.1016/j.ejrad.2015.08.004 . [DOI] [PubMed] [Google Scholar]

PLoS One. doi: 10.1371/journal.pone.0312548.r001

Decision Letter 0

Gurinder Kumar

29 Aug 2024

PONE-D-24-28190Analysis of associated malformations in adults with polysplenia syndrome on CTPLOS ONE

Dear Dr. liu,

Thank you for submitting your manuscript to PLOS ONE. After careful consideration, we feel that it has merit but does not fully meet PLOS ONE’s publication criteria as it currently stands. Therefore, we invite you to submit a revised version of the manuscript that addresses the points raised during the review process.

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Reviewers' comments:

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Comments to the Author

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Reviewer #1: Yes

Reviewer #2: Yes

Reviewer #3: Yes

Reviewer #4: Yes

**********

2. Has the statistical analysis been performed appropriately and rigorously?

Reviewer #1: Yes

Reviewer #2: No

Reviewer #3: Yes

Reviewer #4: N/A

**********

3. Have the authors made all data underlying the findings in their manuscript fully available?

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Reviewer #1: Yes

Reviewer #2: Yes

Reviewer #3: Yes

Reviewer #4: No

**********

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Reviewer #1: Yes

Reviewer #2: Yes

Reviewer #3: Yes

Reviewer #4: Yes

**********

5. Review Comments to the Author

Please use the space provided to explain your answers to the questions above. You may also include additional comments for the author, including concerns about dual publication, research ethics, or publication ethics. (Please upload your review as an attachment if it exceeds 20,000 characters)

Reviewer #1: Rigorously written article by the authors. It is a retrospective series, however the rarity of the condition makes the article useful. Adding percentages of findings in tables would have added to the readability of the article.

Reviewer #2: Dear Author,

Greetingd to you for drafting a study on an important disorder such as the Polysplenia disorder.

I commend your efforts totally.

I request you to please go through these observations at my end.

1. You can shorten the running title as the main title and running title appear to be the same.

2. Keywords may be chosen a bit more appropriately, for example, you may use a keyword like malformations which may be used instead of 'adult' making it a bit more specific and catchy.

3. The sample size being 12, I request you to make a small change in your title if possible, you can call this as a 'pilot' study.

4. In financial disclosure section, you have disclosed that there was no funding prpvided for your study. Under the fundings head, you are mentioning certain project names. Can you please clarify it further on how the projects helped in your study funding? Was it based on collection of patients or something related?

5. In your objective, can you replace the word 'summarise' to 'analytically depict' so that it becomes more measurable and oriented to your study results.

6. If you can have some statistical tests suitaing this quantitative study wherein you can further supplement your results with statisical evidence, it would make your study stronger.

7. Were the examining radiologists blinded from the patients? Were the patients blinded from the examining radiologists? Were both blinded from each other? Were the examining radiologists aware that this sort of study may take place in future subject to their reporting?

You can go through these points and take your time to consider them as well.

Regards.

Reviewer #3: Thank you for providing me the opportunity to review this original article. This article presents an interesting and rare appearance of polysplenia and associated anomalies. A few corrections are required for further processing which have been described in the word file in the comment section.

Reviewer #4: With 12 cases, the topic should be better under a case review series rather than a research article.

Author/s should describe the spectrum of heterotaxy or situs ambiguus syndromes in a small paragraph in the discussion introduction.

The author/s should mention in a concise paragraph the need for symptomatic management in the patients, depending on the type of associated anomaly, along with the importance of frequent follow-up with a multidisciplinary team.

Fig 1l Duodenal duodenum anterior portal vein (PDPV) - correct the terminology

**********

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Reviewer #1: No

Reviewer #2: Yes: Gaurav Vedprakash Mishra

Reviewer #3: Yes: Anshul Sood

Reviewer #4: Yes: Dr Swati Goyal

**********

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Attachment

Submitted filename: PONE-D-24-28190_reviewer.docx

pone.0312548.s001.docx^{(1.3MB, docx)}

PLoS One. 2024 Dec 20;19(12):e0312548. doi: 10.1371/journal.pone.0312548.r002

Author response to Decision Letter 0

26 Sep 2024

For review comments

Reviewer1：

1. Rigorously written article by the authors. It is a retrospective series, however the rarity of the condition makes the article useful. Adding percentages of findings in tables would have added to the readability of the article.

Answer：We had added the percentages of findings in table.

Reviewer2:

1. You can shorten the running title as the main title and running title appear to be the same.

Answer：We think this is an excellent suggestion. We have simplified the running title to “Malformations in Adults with Polysplenia on CT”.

2. Keywords may be chosen a bit more appropriately, for example, you may use a keyword like malformations which may be used instead of ＂adult＂ making it a bit more specific and catchy.

Answer：Thanks for your suggestion. We have replaced the keyword “adult” with “malformations”.

3. The sample size being 12, I request you to make a small change in your title if possible, you can call this as a ＂pilot＂ study.

Answer：We agree with your suggestion. We have added “A Pilot Study” in Title.

Answer：The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript. We had added this sentence in Statement.

5. In your objective, can you replace the word ＂summarise＂ to ＂analytically depict＂ so that it becomes more measurable and oriented to your study results.

Answer：As suggested by the reviewer, we have replaced the word “summarise” with “analytically depict”.

6. If you can have some statistical tests suitaing this quantitative study wherein you can further supplement your results with statisical evidence, it would make your study stronger.

Answer：Thank you for the valuable suggestions. We had added the percentages of findings in result and table 2.

Answer：The study is a retrospective study. We retrospectively analyzed the CT imaging data of twelve adults of PS to summarize the concomitant deformities in adults of PS with the aim of improving the understanding of concomitant deformities of PS, avoiding misdiagnosis of anatomical variants as lesions and causing unnecessary surgical injuries.

Reviewer3:

1. Kindly add full form of CT in the title.

Answer: Thank you for the valuable suggestions. As suggested by the reviewer, we have added the full form of CT (Computed Tomography) in the title.

2. Kindly add an ＂s＂after ＂patient＂.

Answer: Thank you for your detailed review. We have made the necessary corrections to the term “patient”.

3. Kindly correctly label the figure 1b and figure 1c.

Answer: Thanks for this comment. We have corrected the label of the figure 1b and figure 1c.

4. “observed” would be a better term than “affected”.

Answer: Thanks for your suggestion. We have replaced “affected” with “observed” as suggested.

5. Throughout the manuscript, kindly use full form of the acronym when it is used as a first word of the sentence.

Answer: As suggested by the reviewer, we have changed the abbreviations at the beginning of paragraphs throughout the manuscript to their full forms.

6. Kindly use a widely accepted term of enhancement for describing the pattern of enhancement. Something like Zebra pattern.

Answer: We appreciate your suggestion and have made the necessary changes, replacing “blotchy” enhancement with “Zebra pattern” enhancement.

7. Pancreas would suffice.

Answer: Your suggestion is greatly appreciated. We have made the change “Pancreases” to “Pancreas”.

8. Rewrite the sentence ＂Since the normal pancreas...＂ for clarity.

Answer: We sincerely thank the reviewer for careful reading. As suggested by the reviewer, we have revised the sentence to “The pancreas is formed by the fusion of ventral and dorsal pancreatic buds; the ventral pancreatic buds give rise to the uncinate process and head, and the dorsal pancreatic buds give rise to the body and tail [14]. When the pancreas is dorsally underdeveloped, dysplasia of the body and tail of the pancreas, occurs which mostly manifests as a short pancreas [14].”.

9. Kandly identify the site of gall bladder.

Answer: Thank you for your suggestion. We had checked the site of gall bladder.

10. This statement contradicts the statement in the results section and image 1m. Kindly confirm and rewrite.

Answer: We had made a consistent statement for this finding in our paper.

11. Conclusion should be a separate heading and not a subheading. Kindly rectify.

Answer: As suggested by the reviewer, we have revised the heading of Conclusion.

12. Kindly elaborate what does the white arrow in the part (d) indicates.

Answer: It showed the hirizontal liver and biliary stones. To avoid misunderstanding, we had deleted this arrow.

13. Kindly enlarge the dotted arrow in the figure 1k and figure 1m.

Answer: We had enlarged the dotted arrows in the fig 1k and fig 1m.

14. Kindly add arrows to the image 1p.

Answer: We had added the arrows for image 1p.

15. Kindly link the table 2 in the text.

Answer: We had linked the table 2 in the text.

Reviewer4:

1. With 12 cases, the topic should be better under a case review series rather than a research article.

Answer: Thank you for the helpful suggestion. We had added the A PILOT STUDY in Title.

2. Author/s should describe the spectrum of heterotaxy or situs ambiguus syndromes in a small paragraph in the discussion introduction.

Answer: We had added some sentences about heterotaxy or situs ambiguous in the first paragraph in discussion.

3. The author/s should mention in a concise paragraph the need for symptomatic management in the patients, depending on the type of associated anomaly, along with the importance of frequent follow-up with a multidisciplinary team.

Answer: We had added a concise paragraph, “Being familiar with the occurrence of malformations associated with PS is conducive to avoiding misdiagnosis and mistreatment. Because the malformations involve multiple systems, multidisciplinary consultation is more conducive to the evaluation of this disease.”, as last paragraph.

4.Fig 1l Duodenal duodenum anterior portal vein (PDPV) - correct the terminology

Answer: Thank you for your suggestion. We have corrected ＂Duodenal duodenum anterior portal vein (PDPV)＂ to ＂Preduodenal portal vein (PDPV)＂.

Attachment

Submitted filename: point-by-point responses.docx

pone.0312548.s002.docx^{(26.3KB, docx)}

PLoS One. doi: 10.1371/journal.pone.0312548.r003

Decision Letter 1

Gurinder Kumar

9 Oct 2024

Analysis of associated malformations by computed tomography in adults with polysplenia syndrome: A Pilot Study

PONE-D-24-28190R1

Dear Dr. liu,

We’re pleased to inform you that your manuscript has been judged scientifically suitable for publication and will be formally accepted for publication once it meets all outstanding technical requirements.

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Kind regards,

Gurinder Kumar, MD

Academic Editor

PLOS ONE

Additional Editor Comments (optional):

Reviewers' comments:

Reviewer's Responses to Questions

Comments to the Author

1. If the authors have adequately addressed your comments raised in a previous round of review and you feel that this manuscript is now acceptable for publication, you may indicate that here to bypass the “Comments to the Author” section, enter your conflict of interest statement in the “Confidential to Editor” section, and submit your "Accept" recommendation.

Reviewer #2: All comments have been addressed

Reviewer #3: All comments have been addressed

**********

2. Is the manuscript technically sound, and do the data support the conclusions?

Reviewer #2: Yes

Reviewer #3: Yes

**********

3. Has the statistical analysis been performed appropriately and rigorously?

Reviewer #2: Yes

Reviewer #3: Yes

**********

4. Have the authors made all data underlying the findings in their manuscript fully available?

Reviewer #2: Yes

Reviewer #3: Yes

**********

5. Is the manuscript presented in an intelligible fashion and written in standard English?

Reviewer #2: Yes

Reviewer #3: Yes

**********

6. Review Comments to the Author

Reviewer #2: Dear authors, it is a nice effort by you to carry up this draft in such a manner.

my comments have been satisfactorily addressed.

Good luck to all of you.

Reviewer #3: The recommended changes have been incorporated in the manuscript.

**********

7. PLOS authors have the option to publish the peer review history of their article (what does this mean?). If published, this will include your full peer review and any attached files.

If you choose “no”, your identity will remain anonymous but your review may still be made public.

Do you want your identity to be public for this peer review? For information about this choice, including consent withdrawal, please see our Privacy Policy.

Reviewer #2: Yes: Gaurav Vedprakash Mishra

Reviewer #3: Yes: Anshul Sood

**********

PLoS One. doi: 10.1371/journal.pone.0312548.r004

Acceptance letter

Gurinder Kumar

10 Dec 2024

PONE-D-24-28190R1

PLOS ONE

Dear Dr. liu,

I'm pleased to inform you that your manuscript has been deemed suitable for publication in PLOS ONE. Congratulations! Your manuscript is now being handed over to our production team.

At this stage, our production department will prepare your paper for publication. This includes ensuring the following:

* All references, tables, and figures are properly cited

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If revisions are needed, the production department will contact you directly to resolve them. If no revisions are needed, you will receive an email when the publication date has been set. At this time, we do not offer pre-publication proofs to authors during production of the accepted work. Please keep in mind that we are working through a large volume of accepted articles, so please give us a few weeks to review your paper and let you know the next and final steps.

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on behalf of

Dr. Gurinder Kumar

Academic Editor

PLOS ONE

Associated Data

This section collects any data citations, data availability statements, or supplementary materials included in this article.

Supplementary Materials

Attachment

Submitted filename: PONE-D-24-28190_reviewer.docx

pone.0312548.s001.docx^{(1.3MB, docx)}

Attachment

Submitted filename: point-by-point responses.docx

pone.0312548.s002.docx^{(26.3KB, docx)}

Data Availability Statement

All relevant data are within the manuscript.

[pone.0312548.ref001] 1.Abdullah RA, Alharbi MA, Alnutaifi FM, Faqih AI, Alduraibi KM, Alharbi AS. Polysplenia Syndrome Complicated by Splenic Infarction: A Report of a Rare Case. Cureus. 2023;15(5):e38589. Epub 2023/06/07. doi: 10.7759/cureus.38589 . [DOI] [PMC free article] [PubMed] [Google Scholar]

[pone.0312548.ref002] 2.Frescura C, Ho SY, Giordano M, Thiene G. Isomerism of the atrial appendages: morphology and terminology. Cardiovasc Pathol. 2020;47:107205. Epub 20200116. doi: 10.1016/j.carpath.2020.107205 . [DOI] [PubMed] [Google Scholar]

[pone.0312548.ref003] 3.Malki MC, Outznit M, Mechhor S, Bouibaouen B, Nkurunziza L, Bacha HE, et al. Polysplenia syndrome in adulthood: a case report. Pan Afr Med J. 2022;41:67. Epub 2022/04/05. doi: 10.11604/pamj.2022.41.67.29014 . [DOI] [PMC free article] [PubMed] [Google Scholar]

[pone.0312548.ref004] 4.Rameshbabu CS, Gupta KK, Qasim M, Gupta OP. Heterotaxy Polysplenia Syndrome In An Adult With Unique Vascular Anomalies: Case Report With Review Of Literature. J Radiol Case Rep. 2015;9(7):22–37. Epub 2015/12/03. doi: 10.3941/jrcr.v9i7.2210 . [DOI] [PMC free article] [PubMed] [Google Scholar]

[pone.0312548.ref005] 5.Mountassir ME, Borahma M, Benelbarhdadi I, Lagdali N, Hosni A, Ajana FZ. Polysplenia syndrome revealed in adulthood by pancreatic and vascular malformations: a case report. Pan Afr Med J. 2022;43:77. Epub 2023/01/03. doi: 10.11604/pamj.2022.43.77.31496 . [DOI] [PMC free article] [PubMed] [Google Scholar]

[pone.0312548.ref006] 6.Tawfik AM, Batouty NM, Zaky MM, Eladalany MA, Elmokadem AH. Polysplenia syndrome: a review of the relationship with viscero-atrial situs and the spectrum of extra-cardiac anomalies. Surg Radiol Anat. 2013;35(8):647–53. Epub 2013/03/20. doi: 10.1007/s00276-013-1100-x . [DOI] [PubMed] [Google Scholar]

[pone.0312548.ref007] 7.Vaughan TJ, Hawkins IF Jr., Elliott LP. Diagnosis of polysplenia syndrome. Radiology. 1971;101(3):511–8. Epub 1971/12/01. doi: 10.1148/101.3.511 . [DOI] [PubMed] [Google Scholar]

[pone.0312548.ref008] 8.Wolla CD, Hlavacek AM, Schoepf UJ, Bucher AM, Chowdhury S. Cardiovascular manifestations of heterotaxy and related situs abnormalities assessed with CT angiography. J Cardiovasc Comput Tomogr. 2013;7(6):408–16. Epub 2013/12/18. doi: 10.1016/j.jcct.2013.11.008 . [DOI] [PMC free article] [PubMed] [Google Scholar]

[pone.0312548.ref009] 9.Gayer G, Apter S, Jonas T, Amitai M, Zissin R, Sella T, et al. Polysplenia syndrome detected in adulthood: report of eight cases and review of the literature. Abdom Imaging. 1999;24(2):178–84. doi: 10.1007/s002619900471 . [DOI] [PubMed] [Google Scholar]

[pone.0312548.ref010] 10.Peoples WM, Moller JH, Edwards JE. Polysplenia: a review of 146 cases. Pediatr Cardiol. 1983;4(2):129–37. doi: 10.1007/BF02076338 . [DOI] [PubMed] [Google Scholar]

[pone.0312548.ref011] 11.Low JP, Williams D, Chaganti JR. Polysplenia syndrome with agenesis of the dorsal pancreas and preduodenal portal vein presenting with obstructive jaundice—a case report and literature review. Br J Radiol. 2011;84(1007):e217–20. doi: 10.1259/bjr/27680217 . [DOI] [PMC free article] [PubMed] [Google Scholar]

[pone.0312548.ref012] 12.Kayhan A, Lakadamyali H, Oommen J, Oto A. Polysplenia syndrome accompanied with situs inversus totalis and annular pancreas in an elderly patient. Clin Imaging. 2010;34(6):472–5. Epub 2010/11/26. doi: 10.1016/j.clinimag.2009.11.011 . [DOI] [PubMed] [Google Scholar]

[pone.0312548.ref013] 13.Kobayashi H, Kawamoto S, Tamaki T, Konishi J, Togashi K. Polysplenia associated with semiannular pancreas. Eur Radiol. 2001;11(9):1639–41. Epub 2001/08/21. doi: 10.1007/s003300000757 . [DOI] [PubMed] [Google Scholar]

[pone.0312548.ref014] 14.Maier M, Wiesner W, Mengiardi B. Annular pancreas and agenesis of the dorsal pancreas in a patient with polysplenia syndrome. AJR Am J Roentgenol. 2007;188(2):W150–3. Epub 2007/01/24. doi: 10.2214/AJR.05.1859 . [DOI] [PubMed] [Google Scholar]

[pone.0312548.ref015] 15.Ito H, Ohgi S, Kanno T, Ishibashi T. Heterotaxy syndrome with pancreatic malrotation: CT features. Abdom Imaging. 2003;28(6):856–8. doi: 10.1007/s00261-003-0034-x . [DOI] [PubMed] [Google Scholar]

[pone.0312548.ref016] 16.Burton EC, Olson M, Rooper L. Defects in laterality with emphasis on heterotaxy syndromes with asplenia and polysplenia: an autopsy case series at a single institution. Pediatr Dev Pathol. 2014;17(4):250–64. Epub 20140415. doi: 10.2350/13-11-1406-OA.1 . [DOI] [PubMed] [Google Scholar]

[pone.0312548.ref017] 17.Hadar H, Gadoth N, Herskovitz P, Heifetz M. Short pancreas in polysplenia syndrome. Acta Radiol. 1991;32(4):299–301. Epub 1991/07/01. . [PubMed] [Google Scholar]

[pone.0312548.ref018] 18.Herman TE, Siegel MJ. Polysplenia syndrome with congenital short pancreas. AJR Am J Roentgenol. 1991;156(4):799–800. Epub 1991/04/01. doi: 10.2214/ajr.156.4.2003448 . [DOI] [PubMed] [Google Scholar]

[pone.0312548.ref019] 19.Fulcher AS, Turner MA. Abdominal manifestations of situs anomalies in adults. Radiographics. 2002;22(6):1439–56. doi: 10.1148/rg.226025016 . [DOI] [PubMed] [Google Scholar]

[pone.0312548.ref020] 20.Li ZG, Lin ZC, Mu HY. Polysplenia Syndrome With Splenic and Skeletal Muscle Metastases From Thyroid Carcinoma Evaluated by FDG PET/CT: Case Report and Literature Review: A Care-Compliant Article. Medicine (Baltimore). 2016;95(4):e2532. doi: 10.1097/MD.0000000000002532 . [DOI] [PMC free article] [PubMed] [Google Scholar]

[pone.0312548.ref021] 21.G AL, Kagali NA, M S, Prasad BS. Preduodenal portal vein in adult with polysplenia syndrome revisited with a case report. Indian J Surg. 2014;76(2):137–42. Epub 2014/06/04. doi: 10.1007/s12262-013-0812-9 . [DOI] [PMC free article] [PubMed] [Google Scholar]

[pone.0312548.ref022] 22.Seo HI, Jeon TY, Sim MS, Kim S. Polysplenia syndrome with preduodenal portal vein detected in adults. World J Gastroenterol. 2008;14(41):6418–20. doi: 10.3748/wjg.14.6418 . [DOI] [PMC free article] [PubMed] [Google Scholar]

[pone.0312548.ref023] 23.Salma EH, Najwa A, Mehdi T, Nazik A, Latifa C, Siham EH. Polysplenia Syndrome With Persistent Left Superior Vena Cava: Case Report and Review of the Literature. Glob Pediatr Health. 2022;9:2333794X221127640. Epub 20220927. doi: 10.1177/2333794X221127640 . [DOI] [PMC free article] [PubMed] [Google Scholar]

[pone.0312548.ref024] 24.Lai B, Zhong J, Zhong J. Polysplenia syndrome complicated with posterior nutcracker syndrome. Asian J Surg. 2023;46(12):5460–1. Epub 2023/08/05. doi: 10.1016/j.asjsur.2023.07.115 . [DOI] [PubMed] [Google Scholar]

[pone.0312548.ref025] 25.Montero-Macias R, Krief D, Villefranque V, Kilani M, Castillo-Tunon JM. Anatomy, embryology, and imaging of situs ambiguous with polysplenia and left IVC. Radiol Case Rep. 2023;18(5):1727–32. Epub 20230227. doi: 10.1016/j.radcr.2023.02.005 . [DOI] [PMC free article] [PubMed] [Google Scholar]

[pone.0312548.ref026] 26.Yorita K, Sasaki S, Kawada A, Okazaki M, Yamai H, Uchita K, et al. Hepatoid Adenocarcinoma of the Extrahepatic Bile Duct in a Patient with Polysplenia Syndrome. Intern Med. 2017;56(4):401–7. Epub 2017/02/17. doi: 10.2169/internalmedicine.56.7526 . [DOI] [PMC free article] [PubMed] [Google Scholar]

[pone.0312548.ref027] 27.Mimatsu K, Oida T, Kano H, Kawasaki A, Fukino N, Kida K, et al. Preduodenal portal vein, intestinal malrotation, polysplenia, and interruption of the inferior vena cava: a review of anatomical anomalies associated with gastric cancer. Surg Radiol Anat. 2012;34(2):179–86. Epub 20111112. doi: 10.1007/s00276-011-0894-7 . [DOI] [PubMed] [Google Scholar]

[pone.0312548.ref028] 28.Iwamura T, Shibata N, Haraguchi Y, Hisashi Y, Nishikawa T, Yamada H, et al. Synchronous double cancer of the stomach and rectum with situs inversus totalis and polysplenia syndrome. J Clin Gastroenterol. 2001;33(2):148–53. Epub 2001/07/27. doi: 10.1097/00004836-200108000-00012 . [DOI] [PubMed] [Google Scholar]

[pone.0312548.ref029] 29.Yoneyama H, Kondo C, Yamasaki A, Nakanishi T, Sakai S. Comparison of situs ambiguous patterns between heterotaxy syndromes with polysplenia and asplenia. Eur J Radiol. 2015;84(11):2301–6. Epub 2015/08/26. doi: 10.1016/j.ejrad.2015.08.004 . [DOI] [PubMed] [Google Scholar]

PERMALINK

Analysis of associated malformations by computed tomography in adults with polysplenia syndrome: A pilot study

Xinru Gu

Shuangshuang Xu

Jinghua Chen

Xiaoqin Jiang

Ping Xie

Xiang Fang

Yan Gao

Jian Huang

Kefu Liu

Roles

Abstract

Objective

Materials and methods

Results

Conclusions

Introduction

Materials and methods

Patients

Analysis methods

Results

Medical history

Table 1. Clinical data of 12 patients with polysplenic syndrome.

CT imaging findings

Table 2. The malformations of 12 patients with polysplenic syndrome on CT.

Fig 1.

Discussion

Pathogenesis and survival rates

CT findings

Multiple spleens

Visceral abnormalities

Vascular malformations

Lung anomalies

Cardiac abnormalities

Comorbid tumors

Differential diagnosis

Conclusions

Data Availability

Funding Statement

References

Decision Letter 0

Gurinder Kumar

Roles

Author response to Decision Letter 0

Decision Letter 1

Gurinder Kumar

Roles

Acceptance letter

Gurinder Kumar

Roles

Associated Data

Supplementary Materials

Data Availability Statement

ACTIONS

PERMALINK

RESOURCES

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