British Journal of Radiology (2007) 80, e94-e97
© 2007 British Institute of Radiology
doi: 10.1259/bjr/62059548
Chronic colonic perforation in pseudomyxoma peritonei
L S Naidu, MRCP, FRCR
and
J C Jobling, FRCS, FRCR
Department of Radiology, Nottingham City Hospital, Hucknall Road, Nottingham NG5 1PB, UK
Correspondence: Dr Leena Naidu, Department of Radiology, Leicester Royal Infirmary, University Hospitals of Leicester NHS Trust, Leicester LE1 5WW, UK. E-mail: srinaidu{at}hotmail.com
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Abstract
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Pseudomyxoma peritonei (PP) produces abundant mucoid material from the rupture of low grade ovarian or appendiceal mucinous tumours. The unique pattern of cancer dissemination and intraperitoneal mucous re-distribution makes it a distinct clinical condition. It has diverse presentations, mainly as a result of narrowing of the gastrointestinal tract and entrapment of other viscera by gelatinous mucoid material. Imaging is helpful, but not diagnostic of this condition. We report a patient with pseudomyxoma perotonei who had perforation at the recto-sigmoid junction into a large mucinous cyst adjacent to the sigmoid colon. The patient improved clinically with conservative management, with persisting communication and no adverse symptoms reported at 4 months follow-up.
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Introduction
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Pseudomyxoma peritonei (PP) is an indolent and poorly understood condition characterized by massive intraperitoneal accumulation of mucoid material secondary to rupture of ovarian or appendiceal mucinous tumours [1]. The mucoid material has typical molecular features and accumulates in large volumes at predetermined anatomic sites [2].
A variety of complications can occur as a result of the disease process including adhesions leading to intermittent intestinal obstruction, inguinal hernia, fistula formation and local spread into pleural and peritoneal spaces. Other complications reported include obstructive uropathy, secondary infertility, hernia sac tumours and acromegaly [3]. Spontaneous colonic perforation into a mucinous cystic collection has not been reported before, to the best of our knowledge.
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Case report
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A 72-year-old man came to the emergency department with complaints of abdominal pain and mucoid diarrhoea. 5 years previously, he had presented with a 9 month history of slowly progressing abdominal distension and 3 week history of increasing abdominal pain and bilious vomiting. Clinical examination at that time revealed distended abdomen with no shifting dullness and all laboratory investigations including full blood count, serum amylase, liver function tests, urea and electrolytes were within normal limits. Plain abdominal film showed dilated small bowel loops. Laporatomy revealed copious amounts of mucoid material with a non-resectable friable gelatinous tumour in the region of right iliac fossa. The peritoneum was thickened with a membrane encasing the surrounding intraperitoneal structures. A diagnosis of PP secondary to low grade primary appendicular tumour was made following histopathological examination. Since then he has had three episodes of intermittent bowel obstruction, which required hospital admission but was managed conservatively.
During the current admission, the patient complained of abdominal pain and mucoid diarrhoea. He was pyrexial with temperature of 38.5°C. Clinical examination showed a tender right iliac fossa mass with no guarding. He had raised white cell count and inflammatory markers. A plain abdominal radiograph (Figure 1
) showed multiple small bowel loops displaced superiorly with paucity of bowel gas in the central abdomen.
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Figure 1. Abdominal radiograph showing displaced small bowel loops supero-laterally with paucity of bowel gas in central abdomen.
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The patient became increasingly unwell over the next week with rising inflammatory markers and grew coliform organisms in the blood culture. 10 days after the initial plain abdominal film, a CT abdomen with intravenous and oral contrast (Figures 2 and 3) showed non-enhancing fluid filled cysts scalloping the liver and spleen with a uniform attenuation of 20 Hounsfield units. A large dilated air and fluid filled cavity occupied most of the abdomen and pelvis, compressing the large bowel and displacing the loops of small bowel. A water-soluble contrast colonic enema (Figures 4 and 5) showed an irregularity on the lateral aspect at recto-sigmoid junction with a contrast filled tract (Figure 6) leading to a large cavity with fluid level which opacified slowly, extending from the peritoneal recess on each side to the midline. These features were suggestive of perforation of large bowel at the level of recto-sigmoid junction, communicating with intraperitoneal cystic cavity. The patient was treated conservatively with nil by mouth, intravenous fluids and antibiotics for 2 weeks. He responded and was discharged in a stable condition.
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Figure 2. CT abdomen with intravenous contrast and oral contrast showing non-enhancing fluid filled cysts scalloping liver and spleen.
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Figure 3. CT abdomen showing a large cavity with air-fluid level compressing the large and small bowel.
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Figure 4. Water-soluble contrast enema (early film) showing irregularity of lateral aspect of recto-sigmoid with contrast filled tract. The contrast has also filled the rest of the colon.
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Figure 5. Water-soluble contrast enema (later film) with patient in standing position showing the contrast filling the large air-filled intraperitoneal cavity, which is extending from both peritoneal recesses.
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Figure 6. Early magnified image of contrast enema study with the patient in prone position showing contrast filled tract at recto-sigmoid junction.
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On follow up at 4 months the patient had abdominal distension with no other symptoms and a repeat CT abdomen showed reduction in the volume of the cystic cavity with persistent air-fluid level suggesting a persistent communication.
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Discussion
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The term "pseudomyxoma peritonei" (PP) was coined in 1884 by Werth and is literally interpreted as "false mucinous tumour of peritoneum". This involves progressive accumulation of mucoid material within the peritoneal cavity with mucinous peritoneal and omental deposits. They usually arise from leakage or local spread of intraperitoneal mucinous neoplasms, i.e. primary ovarian and appendiceal adenomas or adenocarcinomas. This mucous contains epithelial cells compared to the mucous from rupture mucocoele. Histopathologically, they are mucin producing cells within well-differentiated columnar epithelial cells of low-grade malignancy [3].
The tumour cells that escape into the free peritoneal cavity do not implant in the immediate vicinity of primary tumour, but are surrounded by fluid as they move along the flow of peritoneal fluid. Unlike carcinoma cells that implant in a random fashion near the point of bowel perforation, these cells accumulate at specific abdominal and pelvic sites, i.e. the pouch of Douglas, right retrohepatic space, left paracolic gutter, and fossa created by ligament of Trietz [4]. Gravity and absorption of peritoneal fluid are determinant factors for such localizations of tumour cells. The most consistent finding at laporatomy is near complete absence of tumour cells on intestinal surfaces. Exceptions being pyloric antrum, ileocaecal valve, the pouch of Douglas and recto-sigmoid colon within the pelvis as these sites are retroperitoneal and not free to move as a result of peristaltic activity [2, 5]. Hence adhesions, narrowing and fistula formation are common at these sites.
Clinically, patients present with non-specific gastrointestinal symptoms. The common clinical features are abdominal distension (40%), bilateral or unilateral ovarian tumours (20%), hernia sac tumours (20%), appendicitis like syndrome (10%) and infertility (10%) [3]. Primary PP rarely causes complications even in the presence of large volume disease. The recurrent disease is more prone to complications due to fibrosis and intestinal adhesions [6]. Reported complications include intestinal obstruction, small bowel entrapment, obstructive hydronephrosis [7], different types of hernias including inguinal, femoral and umbilical, femoral neuropathy and lower limb oedema due to venous obstruction [5, 8]. Isolated case reports of cutaneous fistula, pleuro-peritoneal fistula and chronic perineal fistula have been seen as complications in these patients.
Diagnosis is difficult and is made mainly by explorative laporatomy, which reveals yellowish-grey thick mucinous material encasing the bowel, omentum and the peritoneum. Radiological investigations are helpful but are not diagnostic.
The role of imaging is mainly in pre-operative planning of surgical procedure and in diagnosis of complications and recurrent disease [6]. Plain abdominal radiograph is of little use and shows non-specific features like peripheral displacement of bowels with obliteration of psoas shadows. It can be of use in impending bowel obstruction, which is a late complication of this disease [9]. The contrast media studies are helpful in demonstrating lack of bowel lumen involvement. In PP, the bowel is commonly indented and displaced by huge peritoneal and retroperitoneal cystic cavities or extrinsic tumour. Occasionally intrinsic mucinous tumours and polyps of colonic origin can be ruled out on barium enema [5].
Reported ultrasound findings include non-mobile echogenic ascites, homogeneous tumour deposits, scalloping of liver margins and splenic margins due to peritoneal implants and rarely mucinous vesicles [10]. Ultrasound can also aid in diagnostic paracentesis of less viscous areas. CT is the most widely used modality in the diagnosis as well as to evaluate the extent of disease. Mucinous ascites with attenuation values greater than water, loculi, scalloping of organs and displacement of bowel gas are typical features of PP. Omental thickening and curvilinear calcification are highly suggestive features [8, 11, 12].
MRI demonstrates the same morphological features as shown by CT. T2 weighted images enables differentiation between mucinous and fluid ascites [13]. There are very few published reports on the use of MRI for diagnosis, which is mainly limited by cost and availability.
The prognosis for this condition is guarded. Various treatment modalities including cyto-reductive surgery (peritonectomy), intraperitoneal chemotherapy and debulking surgery have been tried with varying success. Patients undergoing emergency surgery for complications have an adverse outcome. Repeated laporatomy results in a median survival of 2 years with only a small percentage alive at 5 years [14].
In summary, an unusual complication of PP, namely perforation of large bowel into a large mucinous cavity, is reported. Persisting gas with spontaneous decompression of cystic cavity indicates a chronic communication with the bowel.
Received for publication April 5, 2005.
Revision received June 10, 2005.
Accepted for publication July 11, 2005.
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Abstract
Introduction
