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Discussion |
A 21-month-old male child presented with malnutrition and painless abdominal masses. The masses were provisionally diagnosed as being abdominal lymphoma. Pre-operative investigations did not establish any other cause. The diagnosis of primary gastrointestinal aspergilloma was obtained only post-operatively by histo-pathology and tissue culture. Following surgery, the tumour grew rapidly and massively despite intravenous amphotericin-B, in the recommended doses. The tumour caused recurrent intestinal obstruction which necessitated multiple extensive surgical excisions. The patient finally died due to sepsis and gastrointestinal bleeding. We believe this to be the first description of a primary gastrointestinal aspergilloma with aggressive local infiltration in a non-neutropenic child.
Key words: aspergilloma, aspergillosis, fungus and gastrointestinal, granuloma
J.R.Coll.Surg.Edinb., 45,October 2000, 335-338
Aspergillus infection most commonly involves the lungs and paranasal sinuses but less frequently involves extrapulmonary sites such as the skin, brain, gastrointestinal tract, heart and kidney.1-4 Most of the reports indicate that aspergillosis is an opportunistic infection, especially in immunosuppressed and in cases with AIDS.5 However, it is reported that approximately 25% of patients with aspergillus tracheo-bronchitis are non-immunocompromised.6
Bowel involvement in disseminated aspergillosis is said to be only a part of a coexisting predominant lesion in other organs, most commonly the lung.7 However, intestinal infarction, as the initial organ manifestation of disseminated aspergillosis before lung involvement, has been reported in a patient with acute myelogenous leukaemia.8 One report highlights the high incidence (47%) of aspergillosis in the gastrointestinal tract associated with high dose steroid therapy and lung disease.7
Aspergilloma has been reported in the gastrointestinal tract complicating chronic lymphocytic leukaemia.9 Nakamura et al (1992) reported a fatal oesophageal aspergilloma in an adolescent with leukemia.8 Caecal aspergilloma, diagnosed as acute appendicitis, developed in a child with acute leukaemia after a short course of polychemotherapy.10
To date, we have been unable to document in the literature primary gastrointestinal involvement with aspergilloma, without any secondary focus, in a non-neutropaenic child. We present a case of gastrointestinal aspergilloma which did not respond to either medical or surgical treatment.
A 21-month-old Saudi male child presented with diarrhoea for 3 days. Previously, the child had constipation, abdominal distension, anorexia and weight loss for 45 days prior to admission. On examination, the child looked malnourished, slightly pale and weighed 8.3kg; his height was 74cm. The abdomen was distended but not tender. A huge firm mass was felt in the right flank separate from the kidney and liver. Full blood count showed leukocytosis (31x109/L), Hb 7.6g/dl and platelets 350x109/L. Erythrocyte sedimentation rate was 15mm/first hour. Urine and stool cultures were normal. All relevant investigations were essentially normal. Computed tomography of the abdomen showed a mass attached to the intestine, abdominal wall and right side of the urinary bladder. The provisional diagnosis of gastrointestinal lymphoma was made and the patient was subjected to incisional biopsy of the intestinal mass one week after admission.
During surgery the mass was found to be attached to the ileocecal junction, ascending colon, transverse colon and part of the jejunum. Resection of the involved bowel and primary anastomosis at two sites was achieved. The mass remnants attached to the anterior abdominal wall and the urinary bladder were left alone. Histopathology of the resected specimen showed fungal granulomatous reaction with dichotomously branching septate fungal hyphae (Figures 1, 2). There was penetration of the fungus into the whole bowel thickness sparing the mucosa. Amphotericin-B was given intravenously at 1mg/kg/day and feeding started 10 days post-operatively. On the 14th post-operative day, the patient developed intestinal obstruction necessitating a second laparotomy. A recurrent fungal mass was seen in the mid-ileum. This was resected and primary ileo-ileal anaslomosis was done. Another fungal mass in the midsigmoid was resected. A colostomy and mucous fistula was performed. The mass adherent to the bladder was left alone but the mass involving the anterior abdominal wall was resected and abdominoplasty performed. The child had a second episode of intestinal obstruction 12 days after the second operation. Upper gastrointestinal study with gastrograffin revealed an obstruction at the proximal small bowel. Injection of the contrast material into the colostomy did not visualize the colon. The patient developed sepsis with a coagulopathy state and was treated accordingly. A third laparotomy, due to recurrent intestinal obstruction, was performed 14 days after the second operation. Fungal masses were seen involving the falciform ligament, 15cm of the mid-ileum including the previous anastomosis (Figure 3) and a third mass in the distal sigmoid adherent to the bladder. The falciform ligament was excised together with the mass in the midileum and an ileostomy performed. The previous colostomy and the present ileostomy did not function. The patient developed pancytopenia and a coagulopathy. Blood cultures grew multiple organisms. The patient died due to severe sepsis and gastrointestinal bleeding, 11 days after the third operation (46 days after admission).
Figure 1: A photomicrograph showing a panoramic view of the full thickness of the small intestinal wall showing mixed chronic and granulomatous inflammation mainly in the submucosa, musculasis and serosa sparing the mucosa. (H&E, x80)
Figure 2: A photomicrograph showing a high power view of one of the small intestinal granulomas with a central septated fungal hyphae (H&E; original magnification x180)
Figure 3: Photograph of the resected small intestine with constricted lumen (tip of forceps) and the surrounding previously bisected fungal ball
There are few reports in the literature of gastrointestinal aspergillosis without pulmonary involvement as the initial presentation of disseminated disease.4,1l Tumour-like masses (aspergillomas) in the gastrointestinal tract have been very rarely reported.7,8,9,12 To the best of our knowledge, primary locally infiltrative gastrointestinal aspergilloma in a nonneutropaenic child has never been reported before. The port of entry is usually the lungs and wide dissemination is possible after vascular invasion.9 The gastrointestinal tract may be involved via oesophageal or bowel ulceration, especially in the immunocompromised host.13 However, local invasion has been suggested.8,14 Necrotizing enterocolitis may precede aspergillosis and disruption of mucosal barriers may provide a port of entry for germinating spores.15
The features of gastrointestinal tract involvement are mild anaemia, weight loss, diarrhoea with malabsorption, in the absence of any other cause for the diarrhoea, gastrointestinal haemorrhage and a swinging fever not responding to antibiotics. Occasionally, there may by signs of peritonitis,15 especially if there is bowel perforation or infarction.4,11
Our patient presented with a clinically palpable abdominal mass mimicking an abdominal lymphoma. Intestinal obstruction developed after the first and second laparotomy due to rapidly growing masses in the bowel wall.
Diagnosis of aspergillus infection relies on identifying the organism in cultures or histopathological specimens.9 Extrapulmonary aspergillomas are more difficult to diagnose. In this case, definitive diagnosis was achieved postoperatively by histopathological and microbiological studies. The port of entry of the aspergillus into the bowel wall and the mechanism of such rapid local spread is unclear. Angioinvasion was not seen with special stains in the present case and no evidence of blood dissemination was noted, despite the advanced state of the local disease. In immunocompromised patients, angioinvasion is a noticeable feature in gut aspergillus infection.8,9,15 Previous reports, and this case, support the view that intestinal aspergillosis does not spread by the lymphatic route.
Gastrointestinal haemorrhage is a known complication of aspergillus infection of the gut. This may be due to gut infarction, colonic or duodenal ulceration.15-17 Uncontrollable upper gastrointestinal bleeding due to erosion into the descending aorta, resulting in a rapidly fatal outcome, has been reported in oesophageal aspergilloma.17
The recommended standard therapy is high-dose amphotericin-B (1mg/kg/day).18 Liposomal formulation of amphotericin-B and/ or itraconazole offer additional treatment alternatives.19
In conclusion, gastrointestinal aspergilloma is difficult to diagnose and carries a very poor prognosis.
ACKNOWLEDGEMENT
The authors wish to thank Professor Oluwole J. Ajao, Surgery Department, College of Medicine, King Khalid University, Abha, Saudi Arabia for reading the manuscript.
Copyright date: 17th August 2000
Correspondence to: Dr Ashraf H. M. Ibrahim, Paediatric Surgeon, Asir Central Hospital, P.O.Box: 34, Abha, Saudi Arabia
©2000 The Royal College of Surgeons of Edinburgh, J.R.Coll.Surg.Edinb.