| Abstract|| |
Wandering spleen is a rare clinical entity, characterized by splenic hypermobility that results from elongation or maldevelopment of its suspensary ligaments. The wandering spleen is in constant danger of torsion and infarction. This condition poses a great diagnostic challenge due to lack of awareness and paucity of symptoms. Among adults, it usually occurs in females of childbearing age, the children below ten years of age are other sufferers. The clinical presentation may be acute or chronic; such as asymptomatic abdominal mass, an acute abdomen, or, most commonly, a mass associated with vague abdominal symptoms. Computed tomography and duplex ultrasonography are best diagnostic modalities. The traditional conservative approach carries high risk of infarction leading to splenectomy and postsplenectomy sepsis. Splenopexy is the treatment of choice for all noninfarcted wandering spleens. Splenectomy should only be performed when there is no evidence of splenic blood flow after detorsion of the spleen. The present study, reviews the presentation, course, diagnostic modalities and management options of wandering spleen
Keywords: Wandering spleen, torsion of spleen, splenopexy, splenectomy.
|How to cite this article:|
Qazi SA, Mirza SM, Muhammad AA, Al Arrawi MH, Al-Suhaibani YA. Wandering spleen. Saudi J Gastroenterol 2004;10:1-7
Wandering spleen is rare, even more rarely thought clinical entity that may be totally asymptomatic, yet liable to result in a catastrophic emergency condition of torsion , . The condition is characterized by excessive mobility and displacement of spleen from its normal location in left hypochondrium due to lack of fixation and unduly long splenic pedicle and often, with axial rotation. Wandering spleen is also described as aberrant, floating, displaced, prolapsed, ptotic, dislocated and dystopic spleen. It should be distinguished from ectopic spleen, where splenic tissue develops at an abnormal site, and also from splenosis and accessory spleen. All these conditions denote different etiological and clinical significance , . Hippocrates used the term wandering spleen as early as 1667, when a spleen at an unusual position was found at autopsy  . Until now nearly 500 cases have been reported  . In the present study, we review the literature in the light of our personal experience of wandering spleen in three adult patients; two treated with splenopexy and third had splenectomy.
| Incidence|| |
Wandering spleen is rare. Although exact incidence is impossible to determine, the reported incidence is 0.16%, based on 3853 splenectornies performed for various indications involving all ages , By 1986, 450 cases of wandering spleen were reported in the World literature  A detailed review of the English literature from 1960 to 1992 by Dawson and Roberts documented 148 cases, which included both pediatric and adult cases  . Among adults; females of childbearing age (20-40 years) are the usual victims (70-80%). Children make up 1 /3 of all cases, most of them are below ten years of age , . Male to female ratio is 1: 1, below ten years of age, but for older patients, the ratio is 1:7 , . We encountered three cases, all of them were females between the age of 17-23 years, two were multipara and one unmarried.
| Etiology|| |
The normal spleen is located in the posterior part of the left hypochondrium, is fixed in this position by gastrosplenic and lienorenal ligaments and has little mobility. Failure of development or elongation of these ligaments results in a long splenic mesentery and unusually mobile spleen. The bimodal age incidence favors congenital and acquired etiological factors ,, . The spleen develops in the dorsal mesogastrium, and, with rotation of the gut, it moves posterolaterally to the left. Fusion of dorsal mesogastrium to the posterior abdominal wall and left kidney forms the lienorenal ligament containing tail of pancreas and splenic artery. Failure of fusion produces an abnormally long pedicle. This mechanism is supported by finding the tail of pancreas in the intraperitoneal splenic pedicle, which can get twisted with the spleen resulting in pancreatitis, as was the presentation of one of our cases ,
The gastrosplenic ligament connecting the greater curvature of the stomach to the ventral aspect of the spleen is the other main support. Some additional support is also derived from the phrenocolic ligament, which connects the splenic flexure of the colon to the left hemidiaphargm and invests the lower pole of spleen. Absence of ligaments or failure of these fusions leads to excessive mobility resulting into congenital wandering spleen  . The congenital theory is supported by the presence of early childhood preponderance and the frequent association with other birth defects in 15/66 children under the age of ten years. Also other congenital conditions such as hypermobile colon and prune belly syndrome have association with wandering spleen ,,,,, .
The acquired theory is sustained by the higher incidence in women during the active reproductive years suggesting that pregnancy may contribute to ligamentous lengthening. Laxity of abdominal wall, especially in multipara, could be allied with dependency and elongation of pedicle.
Abell is frequently quoted in support of this argument; he reported 88 females with torsion of the spleen, of whom 72 had obstetric history  . Similar findings were observed in two of our cases. Pregnancy almost certainly plays a role, even though it is not the sole factor , .
Increased weight of the spleen in splenomegaly has been blamed as a cause for lengthening of ligaments. Most wandering spleens are enlarged. In one study, 85% had splenic weight >500 gm and most of the spleens were described as enlarged, congested, and, torsion of pedicle was noted  . For the same reason, malaria has been implicated but is rare in Western countries and even where endemic, its role is far from clear. According to Pearson, in Nigeria 75% of children had malarial splenomegaly but this was not reflected in the number of wandering spleens; others have made similar observation as well , The malarial correlation is weak, although splenomegaly can help in elongation of ligaments, but, instead of predisposing factor, it appears to be an effect of the condition.
| Clinical Presentation|| |
The wandering spleen presents in a variety of ways but the three most common are: (1) Asymptomatic noted as an incidental finding on physical or radiographic examination. (2) Acute abdomen as result of torsion with subsequent infarction. (3) The most common presentation is amass with nonspecific abdominal symptoms or intermittent abdominal discomfort, perhaps due to splenic congestion resulting from torsion and spontaneous detorsion. Buehner and Baker have made similar observation in an extensive review of 133 cases, and same has been observed by other investigators , . In their series of 133 cases, the presence of amass was the most common presentation, 24% of the patients in whom a history was given presented with a subacute or chronic history of a mass. In 16% a mass had been noted for a year or more before diagnosis or treatment or both. The acute pain was the other regular feature, but, 43% of the patients with pain had a subacute or chronic history and 15% had symptoms for a year or more  . Mechanical factors resulting in urinary retention and constipation or symptoms due to pathological disturbances of the spleen such as thrombocytopenia, hypersplenism and lymphoma, have been described  . Other sporadic presentations described are gastric volvolus, variceal hemorrhage  and rarely, acute pancreatitis or necrosis due to torsion of mobile pancreatic tail. , In our three cases, one presented as abdominal catastrophe due to torsion of wandering spleen, another with lower abdominal mass [Figure - 1], and, the third with acute pancreatitis. The latter was also found to have congenital diaphragmatic hernia. To the best of our knowledge, no such previous association (wandering spleen, congenital diaphragmatic hernia and mobile tail of pancreas leading to pancreatitis) has been described in literature.
The splenic torsion may be acute or chronic ,,,,,, Acute torsion presents as a classic acute abdomen and mimics peritonitis, acute appendicitis, twisted ovarian cyst, diverticulitis, cholecystitis and bowel obstruction , . The chronic presentation in the form of abdominal mass, which may be located in any quadrant of abdomen or pelvis can be confused with enlarged kidney or tumors of renal, ovarian, uterine or colonic origin  . A triad of signs from the French literature was first described by Gindrey and Piquard in 1966. It consists of: (a) palpable firm, ovoid mass with notched edge; (b) moving the mass towards the left hypochondrium is painless, but, movement in any other direction may be painful and limited, (c) resonance to percussion in left upper quadrant , . Despite these criteria, a confident preoperative diagnosis on clinical grounds alone is difficult.
| Diagnosis|| |
The hematologic and biochemical investigations are nonspecific. Radiologic imaging such as barium meal or enema provides indirect evidence, but has been replaced by ultrasonography, scintigraphy and CT scanning ,, .
Ultrasonographic findings of a solid mobile mass and absence of spleen from its normal location and doppler ultrasonography of splenic vessels can be used to evaluate flow. Duplex ultrasonography is more specific and has gained popularity, but, it is operator dependent and bowel gases can obscure the findings , . Ultrasonography was diagnostic in two of our three cases. Isotope studies with Tc99m sulfur colloid of the spleen show splenic displacement, as well as the functional status of spleen. In cases of torsion and ischaemia, the spleen is not visualized and only the liver is seen. Scintigraphy gives poor anatomical images and nonvisualization of spleen is nonspecific and nonlocalizing , .
We did not use isotope studies preoperatively but in our opinion, this is very useful for postoperative confirmation of position and viability (functional status) of spleen in cases of splenopexy as shown in [Figure - 2]  . Classically CT manifestations include the absence of the spleen in its normal position, lower abdominal or pelvic mass and whorled appearance of splenic pedicle as shown in [Figure - 3]. The spleen will not be enhanced by intravenous contrast if torsion has occurred and blood supply is lost , .Thick pseudocapsule is a sign of chronic torsion and ischaemia. CT, in addition to demonstration of wandering spleen, also delineates other anatomic relationships and associated pathologies, such as pancreatic tail necrosis and eventration or hernia of the diaphragm, as was observed in one of our cases. CT remains the investigation of choice, and, dynamic studies demonstrate the organ's circulation, and viability of splenic parenchyma , . This information impacts decision making, splenopexy rather than splenectomy, especially in young children. Angiography has been described for diagnosis but is needlessly invasive and not essential  .
| Management|| |
Traditionally, the management of the wandering spleen was splenectomy of infracted spleen, and observation. particularly in children if the spleen is viable with or without symptoms , . This policy was based on the concept that, one can live without a spleen, especially adults. However splenic management has undergone a major change in the last two decades, drifting away from splenectomy to splenopexy ,,,, . This change came as result of better understanding of splenic function and recognition of the fact that its loss is associated with increased risk of overwhelming postsplenectomy sepsis (OPSS). Even the protection afforded by pneumococcal vaccines and daily antibiotic prophylaxis cannot match the presence of an intact, functioning spleen, hence every effort should be made to preserve a wandering spleen and prevent its torsion  . The overall incidence of (OPSS) is 2% in adults and 4% in children, with mortality rates of 1 % and 2% respectively , . The asplenic patients had a 40-fold increased risk of infection and a 17-fold increased risk of fatal sepsis, with mortality rates reaching up to 60% when compared with the population at large. The risk varies with the indications for splenectomy being less in trauma and more in diseased spleen , . Although the risk of OPSS is life long, it is maximum in children below two years of age, and during the first two years after splenectomy , .
Today the recommended treatment of the wandering spleen is operative. Some studies advocate that, in asymptomatic patients, a conservative policy of careful observation can be followed, but, this did not stand the test of time. This was clearly demonstrated in a review of 66 pediatric cases: 39(60%) presented with torsion, 33 had nonviable spleen. Of these, 25(75%) had no previous symptoms. Overall, 50% of the 66 spleens were lost because of ischemia. Thus, the expectant management is unwise even in asymptomatic patients ,, . Splenectomy is indicated for infracted spleen, and sometimes huge splenomegaly precluding splenopexy , . For all other cases splenopexy is the treatment of choice. Although splenopexy has been performed for wandering spleen as early as 1882, it is only with the recent emphasis on splenic preservation, that interest in splenopexy has been revived. , .
The various techniques of splenopexy described in the literature are: (1) Suture of the spleen to diaphragm or abdominal wall either directly or supported by omentum or by dexon mesh , (2) Splenopexy in an extraperitoneal pouch  (3) Disconnecting the gastrcolic ligament, placing the spleen at its anatomical position and then replacing the stomach and colon; suturing the greater curvature of stomach to anterior abdominal wall  .
(4) `Reefing' the splenic pedicle  . (5) Suturing the splenic hilum to the splenic bed  . (6) Mesh splenopexy has been performed recently by the laparoscopic approach  .
In our two cases of splenopexy, we performed prolene mesh splenopexy in one case [Figure - 4], while in the other we used a combination of extraperitoneal pouch, and prolene mesh, as the spleen was too large to be accommodated in the pouch. To the best of our knowledge, this is the first experience of using nonabsorbable prolene mesh, as well as a combination technique for a large spleen, with good result at one year follow up. The success of this may overcome the problem of reoccurrence  and nonavailability of absorbable dexon or polyglactin mesh, as absorbable mesh is used with the view that adhesion formation during mesh dissolution should maintain fixation  . Major complications of splenopexy are rare. The main one is breakdown of sutures with recurrence. Historically, splenopexy for wandering spleen was only detorsion and replacement in its normal location without actual fixation or just simple suture through the capsule. This has generally led to failures ,, . Presently the method of choice for splenopexy is extraperitoneal pouch or mesh splenopexy, both methods have high success rates , . Other complications such as hemorrhage and pseudocapsule formation following suture of spleen are also said not to be a problem. Long-term results of splenopexy have been reported to be good by all authors, although follow up has been limited to less than three years ,, . In elective cases an attempt at splenopexy may still end in splenectomy and preoperative immunization is a wise precaution in all cases. This should be carried out at least two weeks prior to operation to gain the optimal response to capsular antigens.
| Conclusion|| |
The wandering spleen is rare but should be kept in mind, when investigating a "curious" abdominal mass, especially in young females. Ultrasonograhy is a useful screening tool but CT remains the investigation of choice before embarking on treatment. When the diagnosis is made, whether elective or emergent, for a viable wandering spleen, a definitive corrective procedure, such as extraperitoneal pouch or mesh splenopexy, may be regarded as the best treatment option. Unless there are stern contraindications, there is no place for nonoperative management of the wandering spleen.
| References|| |
|1.||Dawson JHM, Roberts NG. Management of the wandering spleen. Aust N Z J Surg 1994; 64: 441-4. |
|2.||Lewis GA, Byrne MP. Wandering spleen. The American Surgeon 1981: 47: 275-7. |
|3.||Buehner M, Baker MS. The wandering spleen. Collective review. Surg Gyne Obst 1992: 175: 373-87. |
|4.||Satydas T, Nasir N, Bradpiece HA. Wandering spleen: case report and literature review. J R Coll of Surg Edin 2002: 47: 512-14. |
|5.||Allen KB, Andrews G. Pediatric wandering spleen- The case for splenopexy: Review of 35 reported cases in the literature J Ped. Surg 1989: 24: 432-3 5. |
|6.||Caracciolo F. Bonatti PL. Castrucci G. Fusco A. Citterio F. Wandering spleen: treatment with colonic displacement. J R Coll of Stir- Edin 1986: 31: 242-4. |
|7.||Desai DC, Hebra A, Davidoff AM, Schnaufer L. Wandering spleen: A challenging diagnosis. Southern J Med 1997; 90: 439-43. |
|8.||Abell I. Wandering spleen with torsion of pedicle. Ann Surg 1933; 98: 722-35. |
|9.||Pearson JB. Torsion of the spleen associated with congenital absence of the left kidney. Br J Surg 1964; 51: 393-5. [PUBMED] |
|10.||Carswell JW. Wandering spleen: I I cases from Uganda. Br J Surg 1974; 61: 495-7. [PUBMED] |
|11.||Daneshgar S, Eras P, Feldman SM, Cacace VA, Federico FN, Levin RH. Bleeding gastric varices and gastric torsion secondary to a wandering spleen. Gastroenterology 1980: 79: 141-3. |
|12.||Sheflin JJ, Lee CM, Kretchmar K. Torsion of wandering spleen and distal pancreas. Am J Radiol 1984; 142: 100. |
|13.||Papakyriacou K, Nicolaou N, Symeonides P. Wandering spleen: a rare emergency condition. Br J Surg 1996: 83: 50. |
|14.||Cainzos M, Amigo F, Porto A, Paulos A, potel J. Acute abdomen caused by torsion of the pedicle in a wandering spleen. HepatoGastroenterol 1993; 40: 78-80. |
|15.||Nemcek AA, Miller FH, Fitzgerald SW. Acute torsion of a wandering spleen: diagnosis by CT and Duplex Doppler and color flow sonography. A J R 1991; 157: 307-9. |
|16.||Gayer G. Zissin R, apter S, Atar E. Portnoy O. Itzchak Y. Ct findings in congenital anomalies of the spleen. Br J Radiol. 2001: 74: 767-72. |
|17.||Stringel G, Soucy P, Mercer S. Torsion of the wandering spleen: splenectomy or splenopexy. J Ped Surg 1982; 17: 373-5. |
|18.||Seashore JH, McIntosh S. Elective splenopexy for wandering spleen. J Ped Surg 1990: 25: 2702. |
|19.||Schmidt SP, Andrews HG, White JJ. The splenic snood: An improved approach for the management of the wandering spleen. J Ped Surg 1992; 27: 1043-4. |
|20.||Jones BJ, Daly M, Delaney PV. Torsion of the spleen managed by splenopexy. Br J Surg 1991; 78: 887-8. [PUBMED] |
|21.||Shaw JHF. Print CG. Postsplenectomy sepsis. Br J Surg 1989; 76: 1074-80. |
|22.||O'Neal BJ. McDonald JC. The risk of sepsis in the asplenic adults. Ann Surg 1981: 194: 775-8. |
|23.||Chaikof EL. McCabe CJ. Fatal overwhelming postsplenectomv infection. Am J Surg 1985: 194: 534-9. |
|24.||Hirose R. Kitano S, Bando T, et al. Laparoscopic splenopexy for pediatric wandering spleen. J Ped Surg 1998; 33: 1571. |
Shabir Ahmad Qazi
P 0 Box 584, Riyadh 11373
Source of Support: None, Conflict of Interest: None
[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]