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T. SATYADAS, N. NASIR and H.A. BRADPIECE
University Department of Surgery, Royal Free Hospital and University Medical School, London, U.K.
J.R.Coll.Edinb., 47, April 2002, 512-514
We report a case of a 17-year-old patient who presented with a 'wandering spleen.' Management options were influenced by her being a Jehovah’s Witness.
Keywords: wandering spleen, splenectomy, splenopexy, Jehovah’s Witness
The spleen is normally situated in the posterior part of the left upper quadrant of the abdomen. It is fixed in this position by the lieno-renal and the gastro-splenic ligaments; the phrenico-colic ligament provides additional support. These ligaments are embryological condensations that take place in the peritoneum and congenital peritoneal anomalies may result in splenic displacement.
A 17-year-old Jehovah’s Witness presented to the gynaecological clinic with a 4-week history of anorexia, abdominal discomfort and lower abdominal swelling. Abdominal examination revealed a smooth 20x10 cm mass, which appeared to be arising from the true pelvis.
Haematological and biochemical investigations were normal and an ultrasound scan of the abdomen and pelvis showed a mass (Figure 1) with a vascular pedicle in the lower abdomen, suggestive of a wandering spleen and she was referred for a surgical opinion. A subsequent computerised tomography (CT) scan of the abdomen and pelvis was reported as showing a solid pelvic tumour (Figure 2). The spleen was not visualised in its normal position.
Figure 1: Ultrasound scan of the abdomen and pelvis showing a mass with a vascular pedicle in the lower abdomen
Figure 2: Computerised tomography (CT) scan of the abdomen and pelvis showing a solid pelvic tumour
At laparotomy a spleen, measuring 15 x 10.5 x 5.5 cm; and weighing 555g, was found lying in the pelvis (Figure3). There was a long vascular pedicle and no lieno-renal ligament. The macroscopic appearance of the spleen was normal. Splenectomy was performed after block tying of the splenic pedicle, flush with the hilum of the spleen, and transfixing with monocryl.
Histological assessment of the excised spleen showed normal splenic tissue. The patient remains well at one-year follow-up.
Figure 3: The excised spleen
Wandering spleen is rare. Fewer than five hundred cases have been reported in the literature. The incidence, based on several large series of splenectomies, is less than 0.5%. 1 A review of the English literature from 1960 to 1992 by Dawson and Roberts (1994), documented 148 cases, which included both paediatric and adult cases. 2
The splenic pedicle is formed by the lieno-renal and gastro-splenic ligaments and contains the splenic artery and vein, and the tail of the pancreas. Embryologically, the splenic anlage is first present in the fifth week of foetal development as a mesodermal proliferation between the two leaves of the dorsal mesogastrium. The spleen is connected to the dorsal body wall in the region of the left kidney by the lieno-renal ligament and to the stomach by the gastro-splenic ligament. If there is incomplete fusion of the dorsal mesogastrium, the spleen may remain on a long pedicle and lie in an ectopic intra-abdominal location. An elongated splenic pedicle is almost always found in a case of a wandering spleen. Malformation or absence of the gastro-splenic and/or lieno-renal ligaments is reported. 3,4
This absence appears to be due to the incomplete fusion of the dorsal mesogastrium. The elongated pedicle in a case of a wandering spleen can predispose to both acute and chronic torsion, with possible infarction.
Clinical presentation can be acute or chronic. In an extensive review 133 cases in the literature by Buehner and Baker (1998), 76 presented with a mass and non-specific abdominal symptoms, 26 patients were asymptomatic, 25 presented with acute abdominal pain, and another six cases had an asymptomatic mass.5 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 in the literature. Torsion of the spleen, whether acute or chronic, with infarction can lead to the development of an “acute abdomen”. Malignant involvement of a wandering spleen is rare and we could find only four reports in the English literature; all four cases had malignant lymphomatous disease.1, 6-8
Wandering spleen is diagnosed relatively more commonly in children than in adults. Dawson and Roberts (1994) found that under the age of 10 years, the sex distribution was even, but for those older than 10 years, females outnumbered males by 7:1.2 Women were found to be affected 13 times more than men in a study of 97 cases.9 There have been suggestions that the flaccidity of the abdominal wall may also play a role in the development of wandering spleen and this may explain the higher incidence in women of childbearing age.
The weight of the normal adult spleen is between 75 - 150 g. Most wandering pelvic spleens are enlarged. In one study, out of the 53 patients for whom the spleen weight was given, 85% had a spleen weight >500 g.9 In another review of 140 cases, most spleens were described as enlarged or congested, and torsion of the pedicle was noted in most reports. 5 Splenomegaly is often secondary to twisting or compression of the pedicle and the resultant congestion. Progressive splenomegaly due to any cause can lead to enhanced splenic mobility. However, splenomegaly generally does not appear to be a predisposing factor to a wandering spleen, but rather is a consequence of the condition.
The clinical diagnosis may be quite difficult and haematological and biochemical investigations may be non-specific. Non-invasive imaging procedures such as ultrasonography, nuclear scintigraphy, CT scanning and magnetic resonance imaging are usually diagnostic. The recommendations in the literature for the management of a wandering spleen are varied. The significant risk of post-splenectomy sepsis supports a conservative approach especially in asymptomatic patients or those with few symptoms.5 In the absence of infarction, thrombosis and hypersplenism, in patients presenting with an acute abdomen, detorsion and splenopexy is a recognised surgical option.10 Different techniques for splenopexy have been described in the literature. Recently, splenopexy has been performed laproscopically.10, 11
There is a view that early splenopexy should replace conservative medical management.2 However, it should be noted that follow-up evaluation data on both open and laparoscopic splenopexy cases are lacking in the literature. In summary, splenopexy is considered to be the optimal treatment for the non-infarcted wandering spleen and splenectomy should be done only when there is evidence of splenic ischaemia after detorsion of the spleen. 2,12,13
In our patient, management options were influenced, to a certain extent, by her being a Jehovah’s Witness. She was very thin and the spleen was clearly palpable above the pelvic brim. There was the possibility that even trivial trauma could lead to splenic rupture. This, coupled with the fact that she would refuse a blood transfusion, was the major indication for splenectomy. Intraoperatively, a long splenic pedicle was found and the lienorenal ligament was absent. Along with this, the tail of the pancreas was malpositioned, indicating a congenital maldevelopment. The spleen was enlarged, weighing over 500 g and was histologically normal. The patient was given the appropriate regimen for prophylaxis against post-splenectomy sepsis syndrome.
1. Kinori I and Rifkin MD. A truly wandering spleen. J Ultrasound Med 1988; 7: 101-5
2. Dawson JH and Roberts NG. Management of the
wandering spleen. Aust N Z J Surg, 1994 ; 64: 441-4
3. Robinson AP. Wandering spleen: Case report and
review. Mt Sinai J Med 1988; 55: 428-34
4. Woodward DA. Torsion of the spleen. Am J Surg, 1967; 114: 953-5
5. Buehner M and Baker MS. The wandering spleen. Surg Gynecol Obstet 1992; 175: 373-87
6. Waldman I and Suissa L. Lymphosarcoma in an
ectopic pelvic spleen. Clin Nucl Med 1978; 3: 417-9
7. Barloon TJ and Lu C. Lymphoma presenting as
an abdominal mass involving an ectopic spleen. Am J
Gastroenterol, 1974; 79: 684-6
8. Dautenhahn LW, Rona G, Saperstein ML, Williams CD and Vermess M. Lymphoma in a pelvic spleen:
CT features J Comput Assist Tomogr 1989; 13:
1081-2
9. Abell I. Wandering spleen with torsion of the pedi
cle. Ann Surg, 1933; 98: 722-35
10. Hirose R, Kitano S, Bando T, Ueda Y, Sato K, Yoshida T, Suenobu S, Kawano T, Izumi T.
Laparoscopic splenopexy for paediatric wandering
spleen. J Paediatr Surg, 1998; 33: 1571-3
11. Cohen MS, Soper NJ, Underwood RA, Quasebarth M, Brunt LM. Laparoscopic splenopexy for wan
dering spleen. Surg Laparosc Endosc, 1998 ; 8:
286-90
12. Steele RD. A torted pelvic spleen. Aust N Z J Surg, 1988; 58: 157-159
13. Rodkey ML, Macknin ML. Pediatric wandering
spleen: case report and
review of the literature. Clin Pediatr(Phila) 1992;
31: 289-94
Copyright: 28 January 2002
Correspondence: T. Satyadas, University Department of Surgery, Royal Free Hospital and University Medical School, London NW3 2QS, U.K.
