54
Journal of the Royal Society of Medicine Volume 83 January 1990
Metastatic carcinoid tumour in association with small bowel ischaemia and infarction
J J Payne-James FRCSa C J de Gara FRCSb D Lovell PhD MRCPathC J J Misiewicz FRCPa N Menzies Gow FRCSb Departments of aSurgery, bHistopathology and cGastroenterology, Central Middlesex Hospital, Acton Lane, London NW1O 7NS Keywords: carcinoid; metastasis; small bowel; ischaemia; infarction
Massive small bowel ischaemia or infarction are rare presentations of metastatic carcinoid tumour. Two cases described emphasize that the prognosis in such cases may be better than expected thereby justifying aggressive surgical treatment. Case reports Case 1: Routine investigations of a 63-year-old woman with abdominal pain and weight loss were inconclusive. At laparotomy the peritoneum was studded with multiple tumour deposits. No primary was identified. The deposits were metastatic carcinoid tumour. Urinary 5 hydroxyindoleacetic acid (5HIAA) was normal. Twelve days postoperatively she developed severe abdominal pain, guarding and absent bowel sounds. At laparotomy the small bowel excluding the proximal 80 cm of jejunum was ischaemic. No mechanical or other cause for the ischaemia was evident. Resection and primary anastomosis was undertaken. There was evidence of thrombosis, embolus or vasculitis. Twentyfour months after the second laparotomy she remains well with no clinical evidence of disease progression. Case 2: A previously fit 45-year-old woman 'collapsed' with abdominal pain of sudden onset. Clinical examination suggested peritonitis. At laparotomy the majority of small bowel (excluding the proximal 50 cm ofjejunum and distal 50 cm of ileum) was gangrenous. Tumour deposits were evident throughout the mesentery, peritoneum and liver. No primary tumour was identified. Histological findings were similar to case 1. Urinary 5HIAA was raised at 92 ymol/24 h. She was discharged at 14 days and remains well and symptom free after 10 months. Discussion Carcinoid tumours account for approximately 20% of all small intestinal neoplasms and are difficult to diagnose tending to present as a result of their complications or serendipitously. Intermittent intestinal obstruction is a
Colonic perforation with a phosphate enema
A M Bell MB BS FRACS Department of Surgery, City Hospital, Nottingham NG5 1PB Keywords: phosphate enema; colonic perforation
Phosphate enemas are in widespread use. There have been several reports of complications associated with their use'-', yet the risks are not widely known. A recent incident has
common presentation, often related to the marked fibrosis of the mesentery that occurs with metastatic carcinoid tumours as a result of the dense desmoplastic or fibroblastic reaction that they may provoke'. Bowel infarction and ischaemia has been recognized as another presenting feature, originally considered to be due to fibrotic mesenteric changes compromising the vasculature, or due to local compression of vessels by tumour. Anthony and Drury2 demonstrated the presence of elastic vascular sclerosis (EVS) - a periadventitial deposition of elastic tissue in 17 of 25 patients with ileal carcinoid, although most were not associated with bowel infarction. Subsequent studies have confirmed the association of EVS with carcinoid tumours. It is suggested that EVS may develop as a result of induction of fibroblasts, or smooth muscle cells by secretory activity ofthe neoplastic deposits'3. It is thought that the presence of EVS may compromise the blood supply to the gut resulting in ischaemia and infarction. EVS was not present in the two cases described. The possibility of ischaemia being caused by intense local vasospasm produced by the secretion of vasoactive amines and peptides from the carcinoid tumour has been suggested, and, although as yet unproven3'4, could account for the findings in these two patients. It has been suggested that bowel infarction most commonly occurs in patients with clinical evidence of carcinoid syndrome although case 2 had an elevated urinary 5HIAA. The presence of long segments of infarcted bowel in patients with evidence of metastatic intra-abdominal neoplasm might imply a very poor prognosis. Successful resection of necrotic bowel and tumour-bearing mesentery has been reported in up to 73% of patients', whilst others have reported a 5-year survival of 20-38% depending on the site of metastases5. Our two patients support the case for surgical extirpation of ischaemic or infarcted bowel when metastatic tumour is found at laparotomy, but no primary tumour is evident, rather than to assume that the prognosis is so poor that no further treatment is undertaken. References 1 Eckhauser FE, Argenta LC, Strodel WE, Wheeler RH, Bull FE, Appelman HD, Thompson NW. Mesenteric angiography, intestinal gangrene and midgut carcinoids. Surgery 1981;90:720-8 2 Anthony PP, Drury RAB. Elastic Vascular sclerosis of mesenteric blood vessels in argentaffin carcinoma. J Clin Pathol 1970; 23:110-18 3 Qizilbash AH. Carcinoid tumors, vascular elastosis, and ischemic disease of the small intestine. Dis Colon Rectum 1977;20:554-60 4 Warner TF, O'Reilly G, Lee GAmcL. Mesenteric occlusive lesion and ileal carcinoids. Cancer 1979;44:758-62 5 Moertel CG, Sauer WG, Dockerty MB, Baggenstoss AH. Life history of carcinoid tumour of the small intestine. Cancer 1961;14:901-12
(Accepted 15 November 1988)
0141-0768/90/
010054-01/$02.00/0 © 1990 The Royal Society of Medicine
lead to this report reiterating the importance of exercising due care with their administration and circumspection with their prescription. Case report A 55-year-old woman was admitted as an emergency to the City Hospital, Nottngham with colicky lower abdominal pain. A life-long history of constipation was elicited. Her abdomen was not tender or distended. Plain abdominal radiographs demonstrated marked faecal loading throughout the colon to the rectum. Initial laboratory investigations revealed a normal biochemical and haematological profile. A phosphate enema was administered. The enema had little effect, a repeat phosphate enema was administered through a rectal tube which had been passed blindly into the recto-sigmoid region.
0141-0768/90/ 010054-02/$02.00/0 © 1990 The Royal Society of Medicine
Journal of the Royal Society of Medicine Volume 83 January 1990
She suddenly developed generalized peritonitis 16 h after the administration of the enema. Her renal function had deteriorated markedly with a serum creatinine rising to 305 mmol/l and serum potassium to 7.2 mmolIl from 60 mmolAI and 4.3 mmol/l, respectively, at admission. At laparotomy there was a small amount offree turbid fluid in the peritoneal cavity with considerable retro-peritoneal oedema extending around the duodenum, involving the transverse mesocolon and the pre-aortic area, and maximally in the sigmoid mesocolon. The colon was empty; there was no diverticulosis. A 1 cm diameter localized perforation of the sigmoid colon was identified just distal to the apex of the sigmoid loop and adjacent to the anterior taenia coli. A limited sigmoid resection was performed with oversewing of the rectal stump and fashioning of an end colostomy. The descending colon was thickened but had a good blood supply. The mucosa was however frankly necrotic as far proximally as could be seen. Postoperatively her renal function deteriorated further with serum creatinine rising to 526 mmol/l before recovering. She developed a marked pulmonary interstitial leak. Liver function tests became diffusely abnormal with a progressive rise in ALP and GGT. Three days after her admission, mineral biochemistry revealed phosphate 1.93 mmol/1 (normal range 0.8-1.4), calcium 2.02 mmol/l, and magnesium 0.69 mmol/l (normal range 0.7-1.0). The serum phosphate subsequently returned to a normal level of 0.99 mmol/l. Histopathological examination of the colon confirmed extensive mucosal necrosis, without obvious cause, and the area of localized perforation. Microbiological culture of a sample of mucosa excised was unremarkable.
Leber's optic neuropathy and mitral valve prolapse
55
Two months later a barium enema was performed per rectum and per stoma prior to successful reanastomosis of the colon; there was no evidence of a stricture. Discussion The sequence of events here was undoubtedly an initial mucosal laceration from the tip of the rectal tube and subsequent extravasation of the hypertonic phosphate solution into the submucosal plane, where it caused mucosal necrosis and localized perforation. A unique feature of this case was the high level of the perforation, induced by the rectal tube. Similar circumstances below the peritoneal reflection are less likely to be diagnosed promptly, leading to rectal gangrene and pelvic sepsis. The severity of this lesion and the biochemical derangement are not in keeping with simple perforation and accompanying sepsis. Clearly enema nozzles must be short and pliable, and phosphate enemata should not be administered when a breach of the mucosa is suspected. References 1 Hool GJ, Bokey EL, Pheils MT. Enema-nozzle injury of the rectum. Med J Aust 1980;1:364 2 Sweeney JL, Hewett P, Riddell P, Hoffmann DC. Rectal gangrene: a complication of phosphate enema. Med J Aust 1986;144:374-5 3 Biberstein M, Parker BA. Enema induced hyperphosphatemia. Am J Med 1985;79:645-6 (Accepted 21 November 1988. Correspondence to Andrew M Bell, Gastroenterology Research Laboratory, Mayo Clinic, 200 First Street, SW, Rochester MN 55905, USA)
I
2
1
2
U~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~
A K Banerjee MRCPa J E MacSweeney MRCPb P G Hykin FRCSb M D Sanders FRCP FRCSb Departments of aCardiology and bMedical Ophthalmology, St Thomas' Hospital, London SEI 7EH Keywords: Leber; electrocardiograph; echocardiograph; mitral valve prolapse
3
1
/w
Case report A previously fit 44-year-old man presented in 1986 with chest pain of sudden onset whilst at work. An electrocardiograph
4
5
6
4
7
PROBAND
E
BILATERAL OPTIC NEUROPATHY
]
UNILATERAL OPTIC NEUROPATHY
Figure 1. Pedigree
Leber's optic neuropathy is characterized by a history of bilateral rapid loss of central vision, first of one eye followed by the second within a few weeks to months. It predominantly affects men in early adult life'. The physical signs classically seen are circumpapillary telangiectatic microangiopathy and swelling of the nerve fibre layer around the optic disc, but no leakage of dye from the disc or papillary regions during fluorescein angiography2. The diagnosis is made on clinical grounds in the presence of a positive family history. The pattern of inheritance has not been fully elucidated but geneological studies have found evidence for a maternal or cytoplasmic mode of inheritance3'5. Leber's disease is associated with cardiovascular symptoms and ECG abnormalities, but mitral valve prolapse has not been previously documented in this condition.
,1V3
2
1
of patient's family
(ECG) taken at the time showed inverted T waves in leads 1, 2, aVL, aVF, V5 and V6 and ST depression in V1-V3 with voltage criteria of left ventricular hypertrophy. The ECG did not change and serial cardiac enzymes were normal. Auscultation was unremarkable but 2D echo confirmed the presence of mitral valve prolapse. Cardiac catheterization confirmed the presence of mitral valve prolapse and revealed normal coronary artery anatomy. He remained well until March 1988, when he presented with a 2 month history of blurred vision in his left eye. He denied any cardiological symptoms. Family history revealed that his mother (Figure 1) suffered bilateral visual loss at the age of 8 years and had documented bilateral optic atrophy. His nephew (Figure 1, III 1) experienced rapid bilateral visual loss at the age of 19 and was diagnosed as having Leber's optic neuropathy. General examination revealed a late soft systolic murmur at the cardiac apex. Ophthalmological examination revealed a visual acuity in the right eye of 6/5 and 6/60 in the left. A left afferent pupillary defect was detected as well as a
0141-0768/90/ 010055-02/$02.00/0 © 1990 The Royal Society of Medicine
Journal of the Royal Society of Medicine Volume 83 January 1990
Metastatic carcinoid tumour in association with small bowel ischaemia and infarction
J J Payne-James FRCSa C J de Gara FRCSb D Lovell PhD MRCPathC J J Misiewicz FRCPa N Menzies Gow FRCSb Departments of aSurgery, bHistopathology and cGastroenterology, Central Middlesex Hospital, Acton Lane, London NW1O 7NS Keywords: carcinoid; metastasis; small bowel; ischaemia; infarction
Massive small bowel ischaemia or infarction are rare presentations of metastatic carcinoid tumour. Two cases described emphasize that the prognosis in such cases may be better than expected thereby justifying aggressive surgical treatment. Case reports Case 1: Routine investigations of a 63-year-old woman with abdominal pain and weight loss were inconclusive. At laparotomy the peritoneum was studded with multiple tumour deposits. No primary was identified. The deposits were metastatic carcinoid tumour. Urinary 5 hydroxyindoleacetic acid (5HIAA) was normal. Twelve days postoperatively she developed severe abdominal pain, guarding and absent bowel sounds. At laparotomy the small bowel excluding the proximal 80 cm of jejunum was ischaemic. No mechanical or other cause for the ischaemia was evident. Resection and primary anastomosis was undertaken. There was evidence of thrombosis, embolus or vasculitis. Twentyfour months after the second laparotomy she remains well with no clinical evidence of disease progression. Case 2: A previously fit 45-year-old woman 'collapsed' with abdominal pain of sudden onset. Clinical examination suggested peritonitis. At laparotomy the majority of small bowel (excluding the proximal 50 cm ofjejunum and distal 50 cm of ileum) was gangrenous. Tumour deposits were evident throughout the mesentery, peritoneum and liver. No primary tumour was identified. Histological findings were similar to case 1. Urinary 5HIAA was raised at 92 ymol/24 h. She was discharged at 14 days and remains well and symptom free after 10 months. Discussion Carcinoid tumours account for approximately 20% of all small intestinal neoplasms and are difficult to diagnose tending to present as a result of their complications or serendipitously. Intermittent intestinal obstruction is a
Colonic perforation with a phosphate enema
A M Bell MB BS FRACS Department of Surgery, City Hospital, Nottingham NG5 1PB Keywords: phosphate enema; colonic perforation
Phosphate enemas are in widespread use. There have been several reports of complications associated with their use'-', yet the risks are not widely known. A recent incident has
common presentation, often related to the marked fibrosis of the mesentery that occurs with metastatic carcinoid tumours as a result of the dense desmoplastic or fibroblastic reaction that they may provoke'. Bowel infarction and ischaemia has been recognized as another presenting feature, originally considered to be due to fibrotic mesenteric changes compromising the vasculature, or due to local compression of vessels by tumour. Anthony and Drury2 demonstrated the presence of elastic vascular sclerosis (EVS) - a periadventitial deposition of elastic tissue in 17 of 25 patients with ileal carcinoid, although most were not associated with bowel infarction. Subsequent studies have confirmed the association of EVS with carcinoid tumours. It is suggested that EVS may develop as a result of induction of fibroblasts, or smooth muscle cells by secretory activity ofthe neoplastic deposits'3. It is thought that the presence of EVS may compromise the blood supply to the gut resulting in ischaemia and infarction. EVS was not present in the two cases described. The possibility of ischaemia being caused by intense local vasospasm produced by the secretion of vasoactive amines and peptides from the carcinoid tumour has been suggested, and, although as yet unproven3'4, could account for the findings in these two patients. It has been suggested that bowel infarction most commonly occurs in patients with clinical evidence of carcinoid syndrome although case 2 had an elevated urinary 5HIAA. The presence of long segments of infarcted bowel in patients with evidence of metastatic intra-abdominal neoplasm might imply a very poor prognosis. Successful resection of necrotic bowel and tumour-bearing mesentery has been reported in up to 73% of patients', whilst others have reported a 5-year survival of 20-38% depending on the site of metastases5. Our two patients support the case for surgical extirpation of ischaemic or infarcted bowel when metastatic tumour is found at laparotomy, but no primary tumour is evident, rather than to assume that the prognosis is so poor that no further treatment is undertaken. References 1 Eckhauser FE, Argenta LC, Strodel WE, Wheeler RH, Bull FE, Appelman HD, Thompson NW. Mesenteric angiography, intestinal gangrene and midgut carcinoids. Surgery 1981;90:720-8 2 Anthony PP, Drury RAB. Elastic Vascular sclerosis of mesenteric blood vessels in argentaffin carcinoma. J Clin Pathol 1970; 23:110-18 3 Qizilbash AH. Carcinoid tumors, vascular elastosis, and ischemic disease of the small intestine. Dis Colon Rectum 1977;20:554-60 4 Warner TF, O'Reilly G, Lee GAmcL. Mesenteric occlusive lesion and ileal carcinoids. Cancer 1979;44:758-62 5 Moertel CG, Sauer WG, Dockerty MB, Baggenstoss AH. Life history of carcinoid tumour of the small intestine. Cancer 1961;14:901-12
(Accepted 15 November 1988)
0141-0768/90/
010054-01/$02.00/0 © 1990 The Royal Society of Medicine
lead to this report reiterating the importance of exercising due care with their administration and circumspection with their prescription. Case report A 55-year-old woman was admitted as an emergency to the City Hospital, Nottngham with colicky lower abdominal pain. A life-long history of constipation was elicited. Her abdomen was not tender or distended. Plain abdominal radiographs demonstrated marked faecal loading throughout the colon to the rectum. Initial laboratory investigations revealed a normal biochemical and haematological profile. A phosphate enema was administered. The enema had little effect, a repeat phosphate enema was administered through a rectal tube which had been passed blindly into the recto-sigmoid region.
0141-0768/90/ 010054-02/$02.00/0 © 1990 The Royal Society of Medicine
Journal of the Royal Society of Medicine Volume 83 January 1990
She suddenly developed generalized peritonitis 16 h after the administration of the enema. Her renal function had deteriorated markedly with a serum creatinine rising to 305 mmol/l and serum potassium to 7.2 mmolIl from 60 mmolAI and 4.3 mmol/l, respectively, at admission. At laparotomy there was a small amount offree turbid fluid in the peritoneal cavity with considerable retro-peritoneal oedema extending around the duodenum, involving the transverse mesocolon and the pre-aortic area, and maximally in the sigmoid mesocolon. The colon was empty; there was no diverticulosis. A 1 cm diameter localized perforation of the sigmoid colon was identified just distal to the apex of the sigmoid loop and adjacent to the anterior taenia coli. A limited sigmoid resection was performed with oversewing of the rectal stump and fashioning of an end colostomy. The descending colon was thickened but had a good blood supply. The mucosa was however frankly necrotic as far proximally as could be seen. Postoperatively her renal function deteriorated further with serum creatinine rising to 526 mmol/l before recovering. She developed a marked pulmonary interstitial leak. Liver function tests became diffusely abnormal with a progressive rise in ALP and GGT. Three days after her admission, mineral biochemistry revealed phosphate 1.93 mmol/1 (normal range 0.8-1.4), calcium 2.02 mmol/l, and magnesium 0.69 mmol/l (normal range 0.7-1.0). The serum phosphate subsequently returned to a normal level of 0.99 mmol/l. Histopathological examination of the colon confirmed extensive mucosal necrosis, without obvious cause, and the area of localized perforation. Microbiological culture of a sample of mucosa excised was unremarkable.
Leber's optic neuropathy and mitral valve prolapse
55
Two months later a barium enema was performed per rectum and per stoma prior to successful reanastomosis of the colon; there was no evidence of a stricture. Discussion The sequence of events here was undoubtedly an initial mucosal laceration from the tip of the rectal tube and subsequent extravasation of the hypertonic phosphate solution into the submucosal plane, where it caused mucosal necrosis and localized perforation. A unique feature of this case was the high level of the perforation, induced by the rectal tube. Similar circumstances below the peritoneal reflection are less likely to be diagnosed promptly, leading to rectal gangrene and pelvic sepsis. The severity of this lesion and the biochemical derangement are not in keeping with simple perforation and accompanying sepsis. Clearly enema nozzles must be short and pliable, and phosphate enemata should not be administered when a breach of the mucosa is suspected. References 1 Hool GJ, Bokey EL, Pheils MT. Enema-nozzle injury of the rectum. Med J Aust 1980;1:364 2 Sweeney JL, Hewett P, Riddell P, Hoffmann DC. Rectal gangrene: a complication of phosphate enema. Med J Aust 1986;144:374-5 3 Biberstein M, Parker BA. Enema induced hyperphosphatemia. Am J Med 1985;79:645-6 (Accepted 21 November 1988. Correspondence to Andrew M Bell, Gastroenterology Research Laboratory, Mayo Clinic, 200 First Street, SW, Rochester MN 55905, USA)
I
2
1
2
U~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~
A K Banerjee MRCPa J E MacSweeney MRCPb P G Hykin FRCSb M D Sanders FRCP FRCSb Departments of aCardiology and bMedical Ophthalmology, St Thomas' Hospital, London SEI 7EH Keywords: Leber; electrocardiograph; echocardiograph; mitral valve prolapse
3
1
/w
Case report A previously fit 44-year-old man presented in 1986 with chest pain of sudden onset whilst at work. An electrocardiograph
4
5
6
4
7
PROBAND
E
BILATERAL OPTIC NEUROPATHY
]
UNILATERAL OPTIC NEUROPATHY
Figure 1. Pedigree
Leber's optic neuropathy is characterized by a history of bilateral rapid loss of central vision, first of one eye followed by the second within a few weeks to months. It predominantly affects men in early adult life'. The physical signs classically seen are circumpapillary telangiectatic microangiopathy and swelling of the nerve fibre layer around the optic disc, but no leakage of dye from the disc or papillary regions during fluorescein angiography2. The diagnosis is made on clinical grounds in the presence of a positive family history. The pattern of inheritance has not been fully elucidated but geneological studies have found evidence for a maternal or cytoplasmic mode of inheritance3'5. Leber's disease is associated with cardiovascular symptoms and ECG abnormalities, but mitral valve prolapse has not been previously documented in this condition.
,1V3
2
1
of patient's family
(ECG) taken at the time showed inverted T waves in leads 1, 2, aVL, aVF, V5 and V6 and ST depression in V1-V3 with voltage criteria of left ventricular hypertrophy. The ECG did not change and serial cardiac enzymes were normal. Auscultation was unremarkable but 2D echo confirmed the presence of mitral valve prolapse. Cardiac catheterization confirmed the presence of mitral valve prolapse and revealed normal coronary artery anatomy. He remained well until March 1988, when he presented with a 2 month history of blurred vision in his left eye. He denied any cardiological symptoms. Family history revealed that his mother (Figure 1) suffered bilateral visual loss at the age of 8 years and had documented bilateral optic atrophy. His nephew (Figure 1, III 1) experienced rapid bilateral visual loss at the age of 19 and was diagnosed as having Leber's optic neuropathy. General examination revealed a late soft systolic murmur at the cardiac apex. Ophthalmological examination revealed a visual acuity in the right eye of 6/5 and 6/60 in the left. A left afferent pupillary defect was detected as well as a
0141-0768/90/ 010055-02/$02.00/0 © 1990 The Royal Society of Medicine
