A wandering spleen is a condition characterized by excessive splenic mobility due to the mal-development or the laxity of its ligaments that leads to migration from its normal anatomical location. This condition renders its long mobile vascular pedicle liable to torsion and occasional infarction. Herein, we report a case of a 27-year-old nulliparous woman who pre- sented with acute abdominal pain and a tender right-sided pelvi-abdominal mass. CT scan of the abdomen and pelvis revealed a torted, infarcted wandering spleen with portal hypertension and mesenteric varices that mandated an emergency splenectomy. Although there are a few reported cases describing the association of wandering spleen with portal hyperten- sion and fundal varices, cases of mesenteric varices are extremely rare. To the best of our knowledge, this case is considered the third reported case of a wandering spleen which was subsequently complicated by portal hypertension and mesenteric varices that was managed effectively with splenectomy. Despite the increasing rate of similar reports in medical lit- INTRODUCTION erature, the actual worldwide incidence is still unknown. It is A wandering spleen, which is also known as an ‘ectopic spleen’, characterized by a bimodal incidence as it generally manifests is a rare condition caused by the improper development or in children and in the third decade of life . weakening of the ligaments that hold the spleen stationary. The aim of this report is to share our experience about the (The congenital mal-development of the dorsal mesogastrium presenting features, and the pitfalls encountered in the man- or the acquired laxities of its ligaments are the possible causes agement of such rare condition. of this condition). Thereby, the spleen is attached at the hilum by only a long vascular pedicle and the organ ‘wanders’ in the lower abdomen or pelvis where it may be mistaken for an CASE REPORT unidentiﬁed abdominal mass. This extra-anatomical ‘ectopic’ position in the abdomen or pelvis (depending on the length of A healthy 27-year-old nulliparous woman reported to the emer- its vascular pedicle) contributes to its variation in clinical pre- gency department with a 2-day history of upper abdominal sentations and makes the diagnosis and management challen- pain associated with repeated vomiting and constipation. ging . Received: March 9, 2018. Accepted: May 4, 2018 Published by Oxford University Press and JSCR Publishing Ltd. All rights reserved. © The Author(s) 2018. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/ licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact email@example.com Downloaded from https://academic.oup.com/jscr/article-abstract/2018/5/rjy107/4999377 by Ed 'DeepDyve' Gillespie user on 21 June 2018 2 B.A. Raﬁe et al. Her symptoms had developed gradually over 2 years prior to devascularisation and ligation of all tributaries was performed her presentation. She used to experience frequent episodes of until hemostasis was secured. upper abdominal pain (mainly in the epigastrium radiating to Apart from paralytic ileus which was managed conserva- both hypochondria) and this pain was relieved by placing her- tively, she was on broad spectrum antibiotic and discharged on self laterally on either sides of the abdomen during the attacks. the 10th postoperative day in a good condition after she The bouts of pain were associated with occasional episodes of received prophylactic triple vaccine against Haemophilus inﬂu- vomiting but she had never presented herself to the emergency enza, Pneumococcus and Meningococcus. Furthermore, she contin- department. A few months prior to her presentation to the hos- ued the prophylactic antibiotic for 1 month postoperatively. pital, the attacks of pain increased and became more frequent. Currently, she is in the third year of her follow up, displaying On the day of her admission, the intensity of pain had increased to the extent of interfering with her movements and activity. The patient had never experienced any abdominal trauma or had never undergone any abdominal surgeries. Upon admission, her vital signs were blood pressure of 105/ 61 mmHg, a pulse rate of 86 beats/min, and there was no evi- dence of fever. An abdominal examination revealed a tender right-sided pelvi-abdominal mass. A digital rectal examination was unremarkable. Laboratory investigation showed a white blood cell count of 15.13 × 10 /UL, (mainly neutrophilia of 90.3%), a hemoglobin level of 9.4 g/dL, and a platelet count of 189 × 10 /UL. The other biochemical parameters including liver enzymes were normal apart from a trace of jaundice, with total Bilirubin of 2.43 mg/dL and direct Bilirubin of 0.45 mg/dL. Chest X-ray showed bowel loops at the splenic fossa (Fig. 1). An initial diagnosis of a torted ovarian cyst was made. A pelvi-abdominal ultrasound revealed normal adnexa but a right-sided soft pelvic-abdominal mass with the absence of a splenic shadow at the left hypochon- drium. Subsequently, CT scan of the abdomen and pelvis revealed a large right-sided infarcted spleen of 30 × 16 × 10 cm (Fig. 2) and a ‘whirl sign’ of the splenic pedicle (Fig. 3). Furthermore, CT scan showed signs of portal hypertension (Fig. 4) associated with mesenteric varices (Fig. 5). The patient subsequently underwent a surgical exploration. An exploratory Laparotomy through a midline incision revealed that the spleen was infarcted and torted around its long vascular pedicle in a 1980° clockwise rotation (11 twists), for which an emergency splenectomy was performed (Fig. 6). In addition, conservative management was elected for the abnor- Figure 2: Computed tomography of the abdomen and pelvis with IV contrast mally dilated and tortuous veins that surrounded the splenic shows a non-enhancing (infarcted) spleen (dotted arrow) that has completely pedicle and the greater curvature of the stomach (Fig. 7). migrated to the right side of the abdomen and pelvis. However, 6 h postoperatively, the patient was taken for re- exploration due to bleeding from these tributaries for which Figure 3: An axial CT of the upper level of the abdomen shows a corkscrew-like soft tissue density (white arrow) indicating twisted splenic vessels surrounded by peritoneal ligaments and fats forming a whirly appearance with alterative Figure 1: Chest x-rays; absence of gastric bubbles at the left hypochondria (LHQ; lucent and dense bands (a whirl sign), is a pathognomonic CT ﬁndings of white arrow) that was replaced with bowel loops. torsion. Downloaded from https://academic.oup.com/jscr/article-abstract/2018/5/rjy107/4999377 by Ed 'DeepDyve' Gillespie user on 21 June 2018 Torsion of a wandering spleen as a cause of portal hypertension and mesenteric varices 3 Figure 6: Intraoperative view of an infarcted wandering spleen with a whirly appearance of splenic pedicles surrounded by peritoneal ligaments and fats. Figure 4: An axial CT shows a widening portal vein 12.7 mm in diameter indic- ating portal hypertension with multiple dilated portal veins seen at the liver parenchyma. Figure 7: Intraoperative view of dilated tortious tributaries of the superior mes- enteric vein adjacent to the handled transverse colon. A wandering spleen can be complicated by hypersplenism, splenic infarction, portal hypertension and/or varices. Although a few cases of a wandering spleen complicated by the fundal varices and left sided portal (sinistrial) hypertension have been reported, but complications of mesenteric varices are con- sidered rare . To the best of our knowledge, this is the third reported case after two cases reported by Zarroug et al.ofa wandering spleen complicated by the mesenteric varices; both were managed successfully by splenectomy. The authors concluded that, in this case the most likely Figure 5: An axial CT shows dilated engorged mesenteric vessels (white arrow) mechanism of portal hypertension with mesenteric varices in indicating mesenteric varices. absence of portal vein thrombosis is the mechanical occlusion of extrahepatic portal vein at the level of superior mesenteric and splenic vein conﬂuence due to splenic torsion and its good health and underwent a laparoscopic cholecystectomy for migration to the right side of the abdomen. Subsequently, this cholelithiasis with an uneventful recovery. led to retrograde long-standing vascular congestion, splenic vein occlusion, and eventually its infarction along with portal hypertension and mesenteric varices. DISCUSSION As the diagnosis of torsion of a wandering spleen is challen- A broad description of clinical manifestation of wandering ging, CT scan imaging showing the characteristic sign of a ‘whirled appearance’ of the splenic pedicle is considered the spleen has been found in the literature. It varies from asymp- tomatic, incidentally discovered conditions such as spontan- preferred modality for diagnosis of this condition . Management of splenic torsion evolved at the beginning of eous atraumatic rupture  or a palpable mass to different degrees of abdominal pain due to acute, subacute, and chronic 20th century, primarily from ‘a splenectomy approach’ toward ‘splenopexies’ using laparoscopic techniques. Currently, sple- torsion and detorsion of its vascular pedicle [4, 5]. Furthermore, the reported degree of torsion ranges from 90° (1/4 twist) to nopexy is the standard surgical procedure for the management of a wandering spleen if it is not infarcted . However, if the 2160° (12 twists) depending on its length and mobility . Downloaded from https://academic.oup.com/jscr/article-abstract/2018/5/rjy107/4999377 by Ed 'DeepDyve' Gillespie user on 21 June 2018 4 B.A. Raﬁe et al. patient develops complications such as those described above, 2. Soleimani M, Mehrabi A, Kashﬁ A, Fonouni H, Buchler MW, a splenectomy may aid in the elimination of varices in addition Kraus TW, et al. Surgical treatment of patients with wan- to symptomatic relief and the subsequent prevention of any dering spleen: report of six cases with a review of the litera- relapse [7, 8]. ture. Surg Today 2007;37:261–69. In conclusion, torsion of a wandering spleen is considered a 3. Moran JC, Shah U, Singer JA. Spontaneous rupture of a wan- life-threatening emergency. Therefore, early recognition and dering spleen: case report and literature review. Curr Surg prompt surgical intervention in symptomatic cases aiming at 2003;60:310–2. preservation of the spleen whenever possible is the primary 4. Faridi MS, Kumar A, Inam L, Shahid R. Wandering spleen— goal in managing such conditions. a diagnostic challenge: case report and review of literature. Malays J Med Sci 2014;21:57–60. 5. Sodhi KS, Saggar K, Sood BP, Sandhu P. Torsion of a wan- CONFLICT OF INTEREST STATEMENT dering spleen: acute abdominal presentation. J Emerg Med The authors declare that we have no conﬂicting interest, and 2003;25:133–7. have not been supported, or funded by any drug company. 6. Maksoud SFA, Swamy N, Khater NH. Tale of a wandering spleen: 1800 degree torsion with infarcted spleen and sec- ondary involvement of liver. J Radiol Case Rep 2014;8: FUNDING 18–26. The authors declare that there was no funding or grant support 7. Sato M, Miyaki Y, Tochikubo J, Onoda T, Shiiya N, Wada H. in this scenario. Laparoscopic splenectomy for a wandering spleen com- plicating gastric varices: report of a case. Surg Case Rep 2015;1:3. PATIENT CONSENT 8. Zarroug AE, Hashim Y, El-Youssef M, Zeidan MM, Moir CR. Consent for taking intraoperative photographs was obtained Wandering spleen as a cause of mesenteric and portal vari- before the patient’s initial operation. ces: a new etiology? J Pediatr Surg 2013;48:E1–4. 9. Priyadrshi RN, Anand U, Kumar B, Prakash V. Torsion in wandering spleen: CT demonstration of whirl sign. Abdom REFERENCES Imaging 2013;38:835–8. 1. Varga I, Galﬁova P, Adamkov M, Danisovic L, Polak S, 10. Anita Magowska. Wandering spleen: a medical enigma, its Kubikova E, et al. Congenital anomalies of the spleen from an natural history and rationalization. World J Surg 2013;37: embryological point of view. Med Sci Monit 2009;15:RA269–276. 545–50. Downloaded from https://academic.oup.com/jscr/article-abstract/2018/5/rjy107/4999377 by Ed 'DeepDyve' Gillespie user on 21 June 2018
Journal of Surgical Case Reports – Oxford University Press
Published: May 18, 2018
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