Wandering spleen is a rare entity that results from the absence or maldevelopment of the ligaments that support the spleen in its normal location. As a result, the spleen is hypermobile and may be predisposed to hilar torsion and subsequent infarction, making it a potentially fatal abdominal emergency. We present a case of a 36-year-old Afghan female who presented with an acute abdomen, and was radiologically and surgically confirmed to have a wandering spleen with torsion and complete infarction. Knowledge of this condition and its radiological findings can play a crucial role in making a correct and timely diagnosis

Abbasi, Summar un Nisa

Khan, Dawar

Khan, Sarim

Khandwala, Kumail

Raza, Rabail

English

eCommons@AKU

eCommons@AKUDepartment of Radiology Medical College, PakistanAugust 2018Torsion of wandering spleen with Infarction.Dawar KhanAga Khan University, dawar.khan@aku.eduKumail KhandwalaAga Khan University, kumail.khandwala@aku.eduSummar un Nisa AbbasiAga Khan UniversitySarim KhanAga Khan UniversityRabail RazaAga Khan University, rabail.raza@aku.eduFollow this and additional works at: https://ecommons.aku.edu/pakistan_fhs_mc_radiolRecommended CitationKhan, D., Khandwala, K., Abbasi, S. N., Khan, S., Raza, R. (2018). Torsion of wandering spleen with Infarction.. Cureus, 10(8), 1-5.Available at: https://ecommons.aku.edu/pakistan_fhs_mc_radiol/316Received 08/15/2018 Review began  08/15/2018 Review ended  08/18/2018 Published 08/21/2018© Copyright 2018Khan et al. This is an open accessarticle distributed under the terms ofthe Creative Commons AttributionLicense CC-BY 3.0., which permitsunrestricted use, distribution, andreproduction in any medium,provided the original author andsource are credited.Torsion of Wandering Spleen withInfarctionDawar B. Khan  , Kumail Khandwala  , Summar-un-nisa Abbasi  , Sarim D. Khan  , RabailRaza 1. Department of Radiology, The Aga Khan University, Karachi. 2. Department of Radiology, The AgaKhan University, Karachi, PAK 3. Department of Radiology, The Aga Khan University, Karachi., Karachi,PAK 4. Medical College, The Aga Khan University, Karachi., Karachi, PAK Corresponding author: Kumail Khandwala, kumail.khandwala@gmail.com Disclosures can be found in Additional Information at the end of the articleAbstractWandering spleen is a rare entity that results from the absence or maldevelopment of theligaments that support the spleen in its normal location. As a result, the spleen is hypermobileand may be predisposed to hilar torsion and subsequent infarction, making it a potentially fatalabdominal emergency. We present a case of a 36-year-old Afghan female who presented withan acute abdomen, and was radiologically and surgically confirmed to have a wandering spleenwith torsion and complete infarction. Knowledge of this condition and its radiological findingscan play a crucial role in making a correct and timely diagnosis.Categories: Emergency Medicine, Radiology, General SurgeryKeywords: wandering spleen, torsion, infarction, hilarIntroductionWandering spleen is a rare clinical entity that results from the absence or maldevelopment ofthe ligaments that support the spleen in its normal location [1]. As a result, the spleen ishypermobile and may be predisposed to hilar torsion and subsequently infarction, making it apotentially fatal emergency. It is an uncommonly encountered condition that mainly affects thepediatric population in one third of cases [2]. In adults, females of reproductive age group aremostly affected, with the cause hypothesized to be hormonal changes during pregnancy leadingto ligamentous laxity [2,3]. We present a case of a 36-year-old female who presented with thiscondition. Knowledge of this entity and its radiological findings can play a crucial role inmaking a correct and timely diagnosis.Case PresentationA 36-year-old multiparous Afghan woman presented to the emergency department with ahistory of abdominal pain and vomiting for one week with sudden increase in intensity of painfor the last three hours. There was no history of fever, prior surgeries or trauma. On physicalexamination, abdominal distension was noted. There was diffuse abdominal tenderness morepronounced in the lower abdomen, but no definite palpable mass was felt.Computed tomography (CT) was performed which revealed absence of the spleen in the leftupper quadrant. An abnormally placed, enlarged spleen was noted in the lower abdomenreaching up to the pelvis. It had an elongated and twisted vascular pedicle. On contrast-enhanced images there was a lack of parenchymal enhancement in the spleen with1 2 3 42 Open Access CaseReport  DOI: 10.7759/cureus.3177How to cite this articleKhan D B, Khandwala K, Abbasi S, et al. (August 21, 2018) Torsion of Wandering Spleen with Infarction.Cureus 10(8): e3177. DOI 10.7759/cureus.3177homogenous low attenuation and peripheral enhancement. The pancreatic tail was alsoinvolved in the torted pedicle (Figures 1, 2).FIGURE 1: Contrast-enhanced computed tomography images.Axial sections showing absence of spleen in its normal position in the left upper quadrant(arrow). An enlarged spleen (asterisk) was found located in the lower abdomen, reaching up tothe pelvis. Low attenuation of the splenic parenchyma was noted with elongated and twistedvascular pedicle (arrowheads). The pancreatic tail was also involved in the twisting of thepedicle as seen in B (arrowhead).2018 Khan et al. Cureus 10(8): e3177. DOI 10.7759/cureus.3177 2 of 5FIGURE 2: Coronal and sagittal computed tomography images.Reformatted images showing abnormal position of the spleen (asterisk). Also seen is thepancreatic tail being pulled along with the vascular pedicle (arrowhead).Non-contrast images confirmed the typical findings of an abnormally located spleen, with ahyperdense splenic pedicle and whorling of the vessels and fat, which were characteristic oftorsion (Figure 3).FIGURE 3: Non-contrast computed tomography images.Axial sections from the abdomen and pelvis showing hyperdense splenic pedicle andcharacteristic whorled appearance of the splenic vessels and fat, representative of torsion ofwandering spleen (arrows).The findings were concluded as wandering spleen with hilar torsion and liquefactive infarction.The patient underwent an exploratory laparotomy. On surgery, the spleen appeared congestedand infarcted, and the splenic vessels were thrombosed. Therefore, total splenectomy wasperformed because of non-viability. The post-operative recovery of the patient was uneventfuland she was discharged after two weeks.DiscussionWandering spleen is a rare entity in which the spleen is hypermobile and ectopic in position,and attached only by an elongated vascular pedicle which becomes prone to torsion. Wanderingspleen is found in less than 0.5% of splenectomies and occurs mainly in children and womenaged 20-40 years [4]. This condition is a result of congenital anomalies in the development ofthe dorsal mesogastrium and the absence or maldevelopment of normal splenic suspensoryligaments resulting in laxity. The splenic ligaments include the gastrolienal and lienorenalligaments which attach the spleen to the stomach and posterior abdominal wall respectively,and the phrenicocolic ligament which supports the spleen inferiorly [1,5].Acquired anomalies have also been described to cause laxity of the supporting ligaments. Thesemay be secondary to abdominal wall weakness, multiple pregnancies, and hormonal changesespecially in reproductive females [3]. Conditions that cause an increase in size of the spleen,like lymphoma, chronic myeloid leukemia and malaria have also been implicated in the2018 Khan et al. Cureus 10(8): e3177. DOI 10.7759/cureus.3177 3 of 5etiology [5]. Both congenital and acquired conditions result in an elongated pedicle, which ispredisposed to torsion and may result in partial or complete infarction because the splenicvessels course within it.Clinical symptomatology is variable and may range from the patient being completelyasymptomatic, presenting with a mobile abdominal lump, or having intermittent abdominalpain because of partial torsion and spontaneous detorsion of the splenic pedicle. Often,patients may also present with an acute abdomen due to complete torsion and infarction. Onexamination, a firm, mobile abdominal mass with characteristic “notched borders” may be felt,but this is always not the case because splenic engorgement may obliterate the splenic notchand therefore a clinical diagnosis is usually tricky [3-5].Radiology plays a crucial role in reaching the correct diagnosis. Plain radiography and bariumstudies are often non-specific and findings may include non-visualization of the splenicshadow or visualization of gas-filled bowel loops in the left upper quadrant (LUQ). A largecentral or lower abdominal soft tissue mass may be seen, and on barium studies there may bedisplacement of splenic flexure with extrinsic impression by the mass [6]. On sonography, thespleen is not present in the splenic bed in the LUQ, and instead a capsulated mass is visualizedin the abdomen or pelvis. This mass may demonstrate a heterogenous or hypoechoicechotexture with reduced or absent intraparenchymal and hilar color flow on Doppler,depending on the degree of torsion [7]. Angiography can also show an abruptly twisted distalsplenic artery at the point of torsion, but it is invasive and therefore not used for diagnosticpurposes [1]. In addition, splenic scintigraphy with heat-damaged red blood cells, which has itsapplications in detecting splenic tissue in cases of splenosis (accessory spleens) and ectopicspleens, has also been used previously for confirming the diagnosis in ambiguous cases [8].Computed tomography (CT), however, is the modality of choice for diagnosing a wanderingspleen when torsion is suspected. CT findings include ectopic position of the spleen, whorledappearance of the splenic pedicle and surrounding fat at the splenic hilum, which may or maynot be accompanied by twisting of the pancreatic tail [8,9]. The spleen may be enlarged due tohypercongestion and there may be minimal or absent post-contrast enhancement of the splenicparenchyma which is usually a sign of infarction [8,10]. Non-contrast CT may reveal lowerattenuation values of the spleen in comparison to the hepatic parenchyma and a hyperdensesplenic pedicle may be seen, which is indicative of the thrombosed splenic vein [10]. Secondaryfindings such as ascites may also be noted. Magnetic resonance imaging (MRI) can help in thediagnosis as well, showing characteristic signals of infarction on both T1- and T2-weightedimages [11] and loss of signal void of the splenic pedicle vessels suggesting thrombosis. Rarecomplications like gastric, pancreatic tail or splenic flexure volvulus and dolichosigmoid of thecolon have also been reported previously in association with wandering spleen [1,5,12,13].Our case showed typical CT findings of a wandering spleen with torsion of its pedicle andcomplete liquefactive infarction. The findings were confirmed during surgery and subsequenthistopathological examination. Until recently, splenectomy has been done for wanderingspleen although many surgeons now recommend open or laparoscopic splenopexy for viablecases. Therefore, partial or total splenectomy should be performed in patients with splenictorsion, in whom infarction and thrombosis of the splenic vessels has occurred [14,15].ConclusionsTorsion and infarction of a wandering spleen is a rare abdominal emergency. Characteristicimaging features on radiological modalities like Doppler and CT scan are crucial in making anaccurate and timely diagnosis. A fair degree of accurate assessment about viability andthrombosis of the splenic vessels can be made on CT scan which may help the surgeon decidethe right mode of treatment.2018 Khan et al. Cureus 10(8): e3177. DOI 10.7759/cureus.3177 4 of 5Additional InformationDisclosuresHuman subjects: Consent was obtained by all participants in this study. Conflicts of interest:In compliance with the ICMJE uniform disclosure form, all authors declare the following:Payment/services info: All authors have declared that no financial support was received fromany organization for the submitted work. Financial relationships: All authors have declaredthat they have no financial relationships at present or within the previous three years with anyorganizations that might have an interest in the submitted work. Other relationships: Allauthors have declared that there are no other relationships or activities that could appear tohave influenced the submitted work.References1. Liu HT, Lau KK: Wandering spleen: an unusual association with gastric volvulus . AJR Am JRoentgenol. 2007, 188:328-330. 10.2214/AJR.05.06722. Ben Ely A, Seguier E, Lotan G, Strauss S, Gayer G: Familial wandering spleen: a first instance . JPediatr Surg. 2008, 43:23-25. 10.1016/j.jpedsurg.2007.12.0773. Yakan S, Telciler KE, Denecli AG: Acute torsion of a wandering spleen causing acuteabdomen. Hong Kong J Emerg Med. 2011, 18:34-36.4. Lam Y, Yuen KK, Chong LC: Acute torsion of a wandering spleen . Hong Kong Med J. 2012,18:160-162.5. El Bouhaddouti H, Lamrani J, Louchi A, et al.: Torsion of a wandering spleen . Saudi JGastroenterol. 2010, 16:288-291. 10.4103%2F1319-3767.706186. Gordon DH, Burrell MI, Levin DC, Mueller CF, Becker JA: Wandering spleen: the radiologicaland clinical spectrum. Radiology. 1977, 125:39-46. 10.1148/125.1.397. Nemeck AA, Miller FH, Fitzgerald SW: Acute torsion of a wandering spleen: diagnosis by CTand Duplex Doppler and color flow sonography. AJR Am J Roentgenol. 1991, 157:307-309.10.2214/ajr.157.2.18538118. Ghahremani S, Sabour M, Ghahremani S, Bazrafshan A, Sadeghi R: A wandering spleendetected by 99mTc-denatured RBC scan. Iran J Nucl Med. 2018, 26:66-67.9. Swischuk LE, Williams JB, John SD: Torsion of wandering spleen: the whorled appearance ofthe splenic pedicle on CT. Pediatr Radiol. 1993, 23:476-477. 10.1007/BF0201245810. Chu J, Li Z, Luo B, Yang J: Wandering spleen with torsion and complete infarction . ActaRadiol. 2011, 52:911-913. 10.1258/ar.2011.10050411. Alam A, Ram MS: Torsion of wandering spleen: diagnosis using magnetic resonance imaging .Med J Armed Forces India. 2006, 62:81-82. 10.1016/S0377-1237(06)80169-312. Sheflin JR, Lee CM, Kretchmar KA: Torsion of wandering spleen and distal pancreas . AJR Am JRoentgenol. 1984, 142:100-101. 10.2214/ajr.142.1.10013. Moorthy K, Deshpande A, Rao P, Vyas S, Supe A: Wandering spleen causing splenic flexurevolvulus and obstruction. Indian J Gastroenterol. 1998, 17:67-68.14. Stringel G, Soucy P, Mercer S: Torsion of the wandering spleen: splenectomy or splenopexy . JPediatr Surg. 1982, 17:373-375. 10.1016/S0022-3468(82)80492-215. Cohen MS, Soper NJ, Underwood RA, Quasebarth M, Brunt LM: Laparoscopic splenopexy forwandering (pelvic) spleen. Surg Laparosc Endosc. 1998, 8:286-290. 10.1097/00019509-199808000-000102018 Khan et al. Cureus 10(8): e3177. DOI 10.7759/cureus.3177 5 of 5

Torsion of wandering spleen with Infarction.

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Torsion of wandering spleen with Infarction.

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