CASE 15356 Published on 27.01.2018

Teaching Case

Section

Abdominal imaging

Case Type

Anatomy and Functional Imaging

Authors

Nicola Tarallo¹, Larissa Nocchi Cardim¹, Valeria Molinelli¹, Anna Leonardi¹, Giorgia Ottini², Stefano Rausei³, Carlo Fugazzola¹

1 Ospedale di Circolo di Varese, University of Insubria, Department of Radiology; Viale Borri 57 21100 Varese, Italy; Email:laranocchi@gmail.com
2 Ospedale di Circolo di Varese, University of Insubria, Department of Anatomical Pathology; Viale Borri 57 21100 Varese, Italy
3 Ospedale di Circolo di Varese, University of Insubria, Department of Surgery; Viale Borri 57 21100 Varese, Italy

Patient

35 years, female

Categories

Area of Interest Abdomen ; Imaging Technique Ultrasound, CT, Experimental ;

Procedure Imaging sequences, Endoscopy ; Special Focus Hyperplasia / Hypertrophy ;

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Clinical History

Hospital admission in 2015 with a history of non-specific abdominal pain. Ultrasound demonstrated an enlarged spleen, located in left hypochondrium. Laboratory findings, serologies and ENA panel were normal, except for a platelet-count of 96, 000/mm3. Because of spontaneous resolution of symptoms, the patient was dismissed without a definitive diagnosis.

Differential Diagnosis List

Torsion of wandering spleen without infarction

Appendicitis

Ovarian diseases

Diverticulitis

Ectopic pregnancy

Imaging Findings

In 2016 US examination showed a pelvic enlarged spleen with normal echogenicity, colour-Doppler US demonstrated patency of splenic artery, spleno-portal confluence and portal vein without flow anomalies (Fig. 1).
To better assess vessels pathways, a CT examination was performed: besides confirming pelvic splenomegaly without ischaemic signs (Fig. 2), it showed a long splenic vascular pedicle formed by the splenic artery in its core, surrounded by multiple ectasic veins, that presented a tortuous and spiraliform layout, indicating the "whirl sign"; the splenic vein was patent only at pancreatic tail level (Figs. 3, 4); furthermore small varicoid veins were visible at gastric wall level (Fig. 3d).
Laparoscopy confirmed imaging findings (Fig. 5); although splenic artery conserved its flow, a splenectomy was performed. Histology showed only spleen congestion without ischaemia (Fig. 6).

Discussion

A. The spleen migrates to a more caudal localisation because of ligaments laxity secondary to congenital or acquired conditions [4-9], resulting in a long pedicle containing splenic vessels [1, 2]. B. Acute or chronic non-specific abdominal or even asymptomatic pain [1, 4, 10]. The major complication is the torsion of long and mobile vascular pedicle [1, 4]. If torsion progresses to more than 360 degrees, usually the arterial blood supply becomes compromised [2, 10, 12]. Repeated episodes of partial torsion and spontaneous detorsion of the splenic pedicle can cause presinusoidal portal hypertension [6]. Hampered venous return determinates splenic enlargement and congestion, resulting in the development of venous outflow collaterals and gastric varices [10, 13]. US, CT and MRI are the main imaging techniques [11]. US is the first approach for vessel flow evaluation [7]; however, there are important limitations, such as bowel gas, difficulty to assess pedicle torsion and hardness in detecting many complications (i.e. varices bleeding) [1, 3, 10]. CT is more accurate [14]; the most specific sign is the "whirl appearance" of the splenic pedicle [4]: it refers to alternate dense and lucent bands in spiral fashion around a central core of vascular structures, respectively caused by thickened peritoneal folds and fat [4, 6]. In addition splenomegaly [4], absent enhancement of splenic parenchyma (in case of ischaemia), hyperdense venous thrombus in the splenic pedicle, “rim-like” enhancement of the capsule can be present [1, 3, 4, 10]. As regards MRI, recent haemorrhagic areas have high signal intensity on T1 and signal increase on T2-weighted images in chronic stage [3, 15]. In our case there are some peculiarities. Firstly, despite splenomegaly and hypersplenism, the spleen was not located in pelvis at the first US evaluation, contrary to the following exams. Secondly, notwithstanding the torsion of vascular pedicle, the splenic artery was patent: this condition is rare, as most of the cases of pedicle torsion reported in literature are associated with spleen infarction [2-6, 10, 11, 14, 16]. Splenopexy is the treatment of choice in paediatric patients or in cases with a minimal degree of torsion, when it is possible to derotate the pedicle and fixate the spleen to abdominal wall or diaphragm with resolvable meshes [3, 10]. In cases of complete pedicle torsion, splenectomy is the gold standard, especially when extensively collateral circulations increase the risk of bleeding [2, 6].

References

[1] Ely AB, Zisin R, Copel L, Vasserman M, Hertz M, Gottlieb P, Gayer G (2006) The wandering spleen: CT findings and possible pitfalls in diagnosis. Clin Radiol 61: 974-58 (PMID: 17018308)

[2] Irak K, Esen I, Keskın M, Emınler AT, Ayyildiz T, Kaya E, Kiyici M, Gürel S, Nak SG, Gülten M, Dolar E (2001) A case of torsion of the wandering spleen presenting as hypersplenism and gastric fundal varices. Turk J Gastroenterol 22 (1): 93-7 (PMID: 21480120)

[3] Maksoud SF, Swamy N, Khater NH (2014) Tale of a wandering spleen: 1800 degree torsion with infarcted spleen and secondary involvement of liver. Radiology Case 8(6): 18-26 (PMID: 25426231)

[4] Priyadarshi RN, Anand U, Kumar B, Prakash V (2013) Torsion in wandering spleen: CT demonstration of whirl sign. Abdom Imaging 38:835-38 (PMID: 22829098)

[5] Desai DC, Hebra A, Davidoff AM, Schnaufer L (1997) Wandering spleen: a challenging diagnosis. South Med J 90(4):439-43 (PMID: 9114840)

[6] Jha AK, Ranjan R, Priyadarshi RN (2017) Wandering spleen and portal hypertension: a vicious interplay. ACG Case Rep J 12;4:e54 (PMID: 28459078)

[7] Chawla S, Boal DK, Dillon PW, Grenko RT (2003) Splenic torsion. Radiographics 23(2):305-8 (PMID: 12640148)

[8] Timpone VM, Lattin GE Jr, Lewis RB, Azuar K, Tubay M, Jesinger RA (2011) Abdominal twists and turns: part 2, solid visceral torsions with pathologic correlation. AJR Am J Roentgenol 197 (1): 97-102 (PMID: 21701016)

[9] Chi Long Ho CL (2014) Wandering spleen with chronic torsion in a patient with thalassemia. Singapore Med J 55(12): e198-e200 (PMID: 25630326)

[10] Rellum R, Risseeuw G, Blaauw Id, Lequin M (2014) Splenorenal collaterals as hallmark for a twisted wandering spleen in a 14-year-old girl with abdominal pain: a case report. Eur J Pediatr Rep 2(1):26-8 (PMID: 25755964)

[11] Karmazyn B, Steinberg R, Gayer G, Grozovski S, Freud E, Kornreich L (2005) Wandering spleen--the challenge of ultrasound diagnosis: report of 7 cases. J Clin Ultrasound 33(9):433-8 (PMID: 16281274)

[12] Deux JF, Salomon L, Barrier A, Callard P, Bazot M (2004) Acute torsion of wandering spleen: MRI findings. Am J Roentgenol 182 (6): 1607-8 (PMID: 15150027)

[13] Sato M, Miyaki Y, Tochikubo J, Onoda T, Shiiya N, Wada H (2015) Laparoscopic splenectomy for a wandering spleen complicating gastric varices: a report case. Surg Case Rep 1(1):3 (PMID: 26943371)

[14] Fujiwara T, Takehara Y, Isoda H, Ichijo K, Tooyama N, Kodaira N, Kitanaka H, Asai T, Kawaguchi K (1995) orsion of the wandering spleen: CT and angiographic appearance. J Comput Assist Tomogr 19(1):84-6 (PMID: 7822555)

[15] Vancauwenberghe T, Snoeckx A, Vanbeckevoort D, Dymarkowski S, Vanhoenacker FM (2015) Imaging of the spleen: what the clinician needs to know. Singapore Med J 56(3): 133-44 (PMID: 25820845)

[16] Misawa T, Yoshida K, Shiba H, Kobayashi S, Yanaga K (2008) Wandering spleen with chronic torsion. Am J Surg 195:504-5 (PMID: 18070724)

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