SPLENOSIS PRESENTING AS PELVIC PAIN
Route Hospital, 8E, Coleraine Road, Ballymoney, Co: Antrim, BT53 6BP. Northern Ireland.
Splenosis occurs after splenic rupture following abdominal trauma. This should be kept in mind as a cause of pain in such patients.
Ectopic splenic tissue results from two aetiologies 1) accessory spleen or spenunculi (congenital); and 2) splenosis (acquired) Splenosis was first documented in dogs in 1883 by Graffini and Tizzani and was not described in humans until 1896 by Albrecht and later Schilling in 1907. The mechanism of autotransplantation of splenic tissue was described by Von Kutner in 1910 and Flat in 1911. The term splenosis was first used to describe this disorder by Buchbinder and Lipkoff in 1939.
A 40 year old lady, para 3 presented to the gynaecology ward with complaints of right sided iliac fossa pain since the past eight weeks. This pain was continuous and constant and radiated down to the thigh and back. The pain was associated with light bleeding per vaginum. There was no fever or any gastro-intestinal symptoms.
She had had a Mirena progesterone coil inserted for menorrhagia about a year and a half ago and had been amenorrhoeic since six months of the insertion. She was sexually active and complained of deep dysparuenia too. She had had a similar episode of pain a year back wherein rupture of a 15 mm cyst in the right ovary was discovered and managed conservatively. In the past she had a history of a splenectomy for a ruptured spleen following a road traffic accident at the age of 18 years. No history of appendicectomy was noted. Physical examination revealed her vital signs to be normal. On palpation, there was tenderness in the right iliac fossa and all other findings were unremarkable. On gynaecological examination, the coil was in situ and uterus was retroverted, mobile and normal in size. There was tenderness in both fornices. WBC count and Urine examination was normal and ultrasound revealed no abnormalities apart from a small amount of fluid in the pouch of douglas and Mirena coil in situ within the uterus.
At laparoscopy, the uterus and both ovaries were normal. There were some adhesions around both adnexae. The pouch of douglas showed clear fluid. There were multiple haemorrhagic polypoidal lesions on the small bowel, the appendix and the pouch of douglas. There was no evidence of infarction or adhesions in these lesions. A biopsy was carried out.Histology confirmed presence of splenic tissue composed of both red and white pulp; surrounded by a fibrous capsule.
Fig 1. Pelvic splenosis
Splenosis is usually a sequel of splenic rupture from abdominal trauma but can be associated with elective splenectomy too. Splenic nodules can be found anywhere in the thoracic cavity, abdominal wall as well as subcutaneously. Although, the true incidence of this event is unknown, a clue is given by Muller and Ruthlin (1995), who ultrasonographically followed patients of post-traumatic splenectomy. They presumed splenosis occurred in one-third of their patients but this was histologically unconfirmed. Fewer than 100 cases of splenosis have been reported in the literature and only a minority of these in gynaecological literature.
Splenosis needs to be distinguished from an accessory spleen, which arises from the side of the dorsal mesogastrium during embryological development. It is commonly located near the gastrosplenic ligament, but in a few cases may be found anywhere within the pancreas, kidney, or in the pelvis as an adnexal mass. Abdominal splenosis occurs throughout the abdominal cavity but the most common sites in order of frequency are the serosal surface of the bowel, the greater omentum, the parietal peritoneum, the serosal surface of the large intestine, the mesentery and the diaphragm.
Most are asymptomatic and discovered by abdominal ultrasound, CT scan or laparotomy during the investigation of another problem. In addition, they can be found during the evaluation of hepatic or renal masses. In a few cases, they may become symptomatic causing abdominal pain, pelvic pain due to infarction, intestinal obstruction, gastrointestinal haemorrhage, an enlarging abdominal mass associated with infection, or hydronephrosis resulting from pressure on the ureter.
Presumed diagnosis can be made using patients history of prior splenectomy or abdominal trauma, absence of siderocytosis and Howell - Jolly bodies on blood smear of patient with history of splenectomy and by scintigraphy using heat damaged Tc-99 labelled autologous red blood cells. The development of high frequency transvaginal scanning has facilitated the diagnosis of relatively small pelvic masses and the advancement of laparoscopic surgical techniques may increase the frequency of diagnosing splenosis. At laparoscopy, these implants, sometimes numerous and spread over the peritoneal cavity are bluish in colour and have no hilus, supplied by local arteries that penetrate their fibrotic capsule. Splenosis may mimic endometriosis. A major difference between these two entities may be the discrepancy between peritoneal spread and the lack of adhesions. They also differ in colour and consistency from malignant lesions and fibroids. Accessory spleen is usually single and is supplied by a branch of the splenic artery.
Therefore the gynaecologist should entertain the diagnosis of splenosis in women with pelvic pain and a history of splenic trauma or splenectomy. Histologic confirmation of splenosis should be verified before proceeding with removal of these implants. However, the most controversial issue is what to do with these splenic nodules, since it is not clear whether they may still provide a protective role against post-splenectomy sepsis. It has been reported that the overall incidence of sepsis and mortality is significantly higher in cases of incidental splenectomy than in post-traumatic splenectomy cases. (Brewster, 1973; Singer, 1973). Thus we may argue that symptomatology is the key issue. When splenosis is diagnosed incidentally in an asymptomatic patient, complete surgical removal is not indicated. Surgical removal is indicated if they are a source of pelvic pain or possible causes of future intestinal obstruction.
Provided malignancy has been ruled out at pre-operative assessment and at the clinical diagnosis phase during endoscopic surgery/resection, laparoscopic resection is a new attractive alternative.
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