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Melanosis of the duodenal mucosa
DISCUSSION The etiology of melanin deposits in the colon is not clearly understood. The origin of the pigment was considered hematogenous by Virchow. 6 Ingestion of heavy metals was incriminated by Williams.? The currently accepted pathogenetic factors are the presence of constipation and ingestion of anthracene containing laxatives 1 by the constipated patient. The pigment has been shown to be present in the cells and macrophages in the colon 8 and in the mononuclear cells in the ileum. 3 Degeneration of mitochondria in the epithelial cells has also been seen and thought to contribute to the formation of the pigment. 8 Our patient did not take any anthracene laxatives although she had constipation. Fecal stasis due to constipation is thought to be a contributory factor for melanosis coli, but our patient had no evidence of any stasis in the colon or duodenum. Whether propranolol, hydrochlorothiazide, sodium tartrate or citrate may cause melanosis is doubtful. There were both extracellular and intracellular deposits of melanin in this patient unlike the previous reported case in which only extracellular deposits were seen s The association of gastric polyps in our patient
seems to be coincidental. While melanosis coli is known to be a benign and potentially reversible condition,' the natural course of melanosis duodeni is not known. We assume that it is a benign condition, causes no symptoms, and is discovered accidentally during panendoscopy.
Melanosis of the duodenal mucosa
of 111 mg, and creatinine of 9.4 mg were noted. Upper gastrointestinal radiography showed a small hiatus hernia and deformed duodenal bulb. Stool guaiac determinations were positive. For this reason, peroral endoscopy was done and revealed a normal esophagus and stomach. The bulb and second portion of the duodenum, as far as could be seen, showed a diffuse pigmentation. Addison's disease and heavy metal exposure were ruled out. Sections from duodenal mucosal biopsy, taken through the endoscope, demonstrated duodenal villi and Brunner's glands. The lamina propria of the villi was packed with irregular cells having a brown, fine dust-like and coarsely granular pigment. The pigment varied in density and depth from field to field and was not seen below the level of the Brunner's glands. The material was negative for iron and showed staining characteristics of true melanin. Staining techniques, used by Dr. Victor Rosen, pathologist at Brotman Memorial Hospital, included hematoxylin and eosin, silver nitrate solution (Fontana), gold chloride solution, sodium thiosulfate solution, and Nuclear Fast Red Kernechtrot solution (Fontana Masson silver method). The pigment was considered not to be pseudomelanin but rather that contained in nevus cells, such as those of a blue nevus as seen in other mucous membranes. Colonoscopy revealed normal mucosa in the large intestine, confirmed on biopsy. The patient was subsequently transferred to Brotman Memorial Hospital for peritoneal dialysis. He expired 3 May 1978 with cerebral encephalopathy and brain stem infarction.
Leonard Breslaw, MD· Century City Medical Plaza Los Angeles, California Bisordi and Kleinman 1 reported a 43-year-old insulindependent diabetic, black man with chronic renal failure whose duodenum presented on endoscopy thickened folds and a peculiar disposition of pigment in the bulb and second part of the duodenum. The rectal mucosa showed no signs of melanosis coli. There was no history of ingestion of cathartics such as cascara. The only medications taken were insulin and aluminum hydroxide gel to lower the serum phosphate. Biopsy sections showed the pigment deposited in the tips of the villi. Special stains confirmed the pigment to be melanin. The pigment was clearly extracellular, collecting in clumps below the epitherial surface. The etiology was obscure. Herein is reported a similar case.
CASE REPORT A 64-year-old black man entered Imperial Hospital in April 1978 with anemia, gastrointestinal bleeding, and hypertension. He had been studied at the University of Southern California-Los Angeles County General Hospital renal clinic since 1959. In 1971 renal failure, hypertension, and congestive failure prompted cystoscopy, which was negative, and a renal biopsy which established a diagnosis of chronic membranous glomerulonephritis. Bilateral absolute glaucoma was recognized in 1973 and eventually resulted in blindness of the right eye. A hemoglobin of 6 g, hematocrit of 19.5%, calcium of 7.6 mg, BUN
* Reprint requests:
Leonard Breslaw, MD, Century City Medical Plaza, Suite 1202, 20BO Century Park East, Los Angeles, California 90067.
VOLUME 26, NO 2, 1980
REFERENCES 1. BOCKUS HL, WILLARD JH, BANK J: Melanosis coli: the etiological significance of the anthracene laxatives: a report of forty-one cases. JAMA 101:1, 1933 2. WITTOESCH JH, JACKMAN RJ, McDONALD jR: Melanosis coli: general review and a study of 887 cases. J Dis Colon Rectum 1:172,1958
3. WON KH, RAMCHAND MB: Melanosis of the ileum: case report and electron microscopic study. Am J Dis 15:57,1970 4. KIMBALL MW: Pseudomelanosis of the esophagus. Gastrointestinal Endoscopy 24:121,1978
5. BIscaRDI WS, KLEINMAN MS: Melanosis duodeni. Gastrointestinal Endoscopy 23(1):37,1976
6. VIRCHOW R: Die Pathologischen Pigmente. Virchows Arch Path Anat 1:379,1847
7. WIlliAMS CT: Black deposits in the large intestine from the presence of mercury. Trans Path Soc (London) 18:111, 1867 8. GHADIALLY FN, PARRY EW: An electron microscope and histochemical study of melanosis coli. J Path Bact 92:313,1966
DISCUSSION The combination of advanced renal disease in a black male patient is common to both Bisordi and 45
Kleinman's case, as well as this one. Diabetes mellitus was not present in this patient. The origin of the melanin remains obscure. There was no stasis as evident by radiography or endoscopy, to contribute to the deposition,
nor was there any history of ingestion of anthracene material, nor was the colon involved in the process.
REFERENCE 1. BISORDI
WM, KLEINMAN MS: Melanosis duodeni. Gastrointes-
tinal Endoscopy 23:37, 1977
Radiographic and endoscopic features of a congenital duodenal diaphragm in an adult A case report and review of the literature A. Turnbull, MD*
S. Kussin, MD M. Bains, MD Gastric and Mixed Tumor Service Department of Surgery and Gastroenterology Service Department of Medicine Memorial Sloan-Kettering Cancer Center New York, New York A congenital diaphragm is an extremely rare cause of duodenal obstruction in adult life and is usually not diagnosed before operation. Halka et al. ' were the first to report the endoscopic features of this deformity, describing a "cervix sign" characterized by a narrow nipple-shaped lumen with circumferential cuffing of the duodenal muco~a at the apex of the bulb giving the appearance of a cervical os. The correct pre-operative diagnosis was also made in the patient reported by Hudson 2 who noted that, in the 11 previously recorded cases, only 1 had been recognized before operation and that a perforated web or diaphragm is probably the only congenital form of intrinsic duodenal obstruction that can remain undiagnosed until adult life. An extensive literature review by Kreig 3 revealed that congenital diaphragms occurred in only 0.8% to 2.5% of all patients with duodenal obstruction or malformation and may be encountered throughout life. The oldest patient described was in the seventh decade, and 29% of patients did not develop symptoms until after 24 years of age. The following adult patient is reported to confirm the characteristic endoscopic and radiographic findings of this condition. The technique used for removal of the diaphragm suggests that subsequent patients might be managed entirely endoscopically with biopsy forceps and electrocautery. CASE REPORT A 56-year-old, obese, white, male physician was admitted in May 1979 with complaints of increasing upper abdominal girth, a weight gain of approximately 20 • Reprint requests: A. Turnbull. MD. Department of Surgery, Memorial Sloan-Kettering Cancer Center. 127S York Avenue. New York, New York 10021.
46
pounds, early satiety, and regurgitation relieved by induced vomiting, all of indefinite duration. His past history revealed no illness pertaining to the gastrointestinal tract; he had enjoyed good health and a generous caloric intake. Barium meal showed massive dilation of the entire stomach with megaduodenum to the level of the superior mesenteric vessels (Figure 1A). There was a large amount of partially digested meat and vegetable matter congealed in his stomach which required several days of nasogastric lavage and drainage before endoscopy could be attempted. During this period, celiac and superior mesenteric arteriography and computerized axial tomography revealed no tumor in the proximal pancreas. The stomach remained distended and patulous despite continuous drainage, and 2 experienced endoscopists were unable to traverse the pyloric canal with a gastroscope to examine the duodenum. Their difficulty was attributed to coiling of the 'scope in the very large antrum, preventing forward movement into the duodenum. After appropriate fluid and electrolyte balance was assured, surgical exploration .revealed a massively distended stomach with extremely thick walls, a very patulous pyloric canal, and a distended duodenum down to the level of the superior mesenteric vessels. Careful examination of the remainder of the abdomen did not demonstrate any other visible or palpable abnormalities. The hepatic flexure of the colon was mobilized, and no obvious extrinsic or intrinsic cause of duodenal compression was found after the entire course of the duodenum was exposed and examined. Because an intrinsic, possibly congenital abnormality was anticipated, the pyloric channel was incised into the proximal duodenum. Digital examination revealed an obstruction with a central orifice at the level of the superior mesenteric vessels which barely admitted the tip of the examining finger. A sterile rigid proctoscope was inserted through the duodenal opening and the diaphragm was visualized. External examination at the site of the obstruction revealed no fibrosis, scarring, or bands. To record these findings, a fiberoptic esophagoscope was inserted which revealed the "cervix sign" and an estimated aperture of 8 mm (Figure 18). The excellent exposure provided by the rigid proctoscope suggested transluminal removal which was done with forward-biting biopsy forceps. Generous pieces of the diaphragm were taken from its 4 quadrants under direct visualization, while an index finger guided the forceps from outside to ensure that only the diaphragm itself was taken. Frozen and subsequent paraffin sections revealed normal duodenal mucosa with fibrosis, glandular atypia, ectasia, and chronic inflammation. After these 4 GASTROINTESTINAL ENDOSCOPY
seems to be coincidental. While melanosis coli is known to be a benign and potentially reversible condition,' the natural course of melanosis duodeni is not known. We assume that it is a benign condition, causes no symptoms, and is discovered accidentally during panendoscopy.
Melanosis of the duodenal mucosa
of 111 mg, and creatinine of 9.4 mg were noted. Upper gastrointestinal radiography showed a small hiatus hernia and deformed duodenal bulb. Stool guaiac determinations were positive. For this reason, peroral endoscopy was done and revealed a normal esophagus and stomach. The bulb and second portion of the duodenum, as far as could be seen, showed a diffuse pigmentation. Addison's disease and heavy metal exposure were ruled out. Sections from duodenal mucosal biopsy, taken through the endoscope, demonstrated duodenal villi and Brunner's glands. The lamina propria of the villi was packed with irregular cells having a brown, fine dust-like and coarsely granular pigment. The pigment varied in density and depth from field to field and was not seen below the level of the Brunner's glands. The material was negative for iron and showed staining characteristics of true melanin. Staining techniques, used by Dr. Victor Rosen, pathologist at Brotman Memorial Hospital, included hematoxylin and eosin, silver nitrate solution (Fontana), gold chloride solution, sodium thiosulfate solution, and Nuclear Fast Red Kernechtrot solution (Fontana Masson silver method). The pigment was considered not to be pseudomelanin but rather that contained in nevus cells, such as those of a blue nevus as seen in other mucous membranes. Colonoscopy revealed normal mucosa in the large intestine, confirmed on biopsy. The patient was subsequently transferred to Brotman Memorial Hospital for peritoneal dialysis. He expired 3 May 1978 with cerebral encephalopathy and brain stem infarction.
Leonard Breslaw, MD· Century City Medical Plaza Los Angeles, California Bisordi and Kleinman 1 reported a 43-year-old insulindependent diabetic, black man with chronic renal failure whose duodenum presented on endoscopy thickened folds and a peculiar disposition of pigment in the bulb and second part of the duodenum. The rectal mucosa showed no signs of melanosis coli. There was no history of ingestion of cathartics such as cascara. The only medications taken were insulin and aluminum hydroxide gel to lower the serum phosphate. Biopsy sections showed the pigment deposited in the tips of the villi. Special stains confirmed the pigment to be melanin. The pigment was clearly extracellular, collecting in clumps below the epitherial surface. The etiology was obscure. Herein is reported a similar case.
CASE REPORT A 64-year-old black man entered Imperial Hospital in April 1978 with anemia, gastrointestinal bleeding, and hypertension. He had been studied at the University of Southern California-Los Angeles County General Hospital renal clinic since 1959. In 1971 renal failure, hypertension, and congestive failure prompted cystoscopy, which was negative, and a renal biopsy which established a diagnosis of chronic membranous glomerulonephritis. Bilateral absolute glaucoma was recognized in 1973 and eventually resulted in blindness of the right eye. A hemoglobin of 6 g, hematocrit of 19.5%, calcium of 7.6 mg, BUN
* Reprint requests:
Leonard Breslaw, MD, Century City Medical Plaza, Suite 1202, 20BO Century Park East, Los Angeles, California 90067.
VOLUME 26, NO 2, 1980
REFERENCES 1. BOCKUS HL, WILLARD JH, BANK J: Melanosis coli: the etiological significance of the anthracene laxatives: a report of forty-one cases. JAMA 101:1, 1933 2. WITTOESCH JH, JACKMAN RJ, McDONALD jR: Melanosis coli: general review and a study of 887 cases. J Dis Colon Rectum 1:172,1958
3. WON KH, RAMCHAND MB: Melanosis of the ileum: case report and electron microscopic study. Am J Dis 15:57,1970 4. KIMBALL MW: Pseudomelanosis of the esophagus. Gastrointestinal Endoscopy 24:121,1978
5. BIscaRDI WS, KLEINMAN MS: Melanosis duodeni. Gastrointestinal Endoscopy 23(1):37,1976
6. VIRCHOW R: Die Pathologischen Pigmente. Virchows Arch Path Anat 1:379,1847
7. WIlliAMS CT: Black deposits in the large intestine from the presence of mercury. Trans Path Soc (London) 18:111, 1867 8. GHADIALLY FN, PARRY EW: An electron microscope and histochemical study of melanosis coli. J Path Bact 92:313,1966
DISCUSSION The combination of advanced renal disease in a black male patient is common to both Bisordi and 45
Kleinman's case, as well as this one. Diabetes mellitus was not present in this patient. The origin of the melanin remains obscure. There was no stasis as evident by radiography or endoscopy, to contribute to the deposition,
nor was there any history of ingestion of anthracene material, nor was the colon involved in the process.
REFERENCE 1. BISORDI
WM, KLEINMAN MS: Melanosis duodeni. Gastrointes-
tinal Endoscopy 23:37, 1977
Radiographic and endoscopic features of a congenital duodenal diaphragm in an adult A case report and review of the literature A. Turnbull, MD*
S. Kussin, MD M. Bains, MD Gastric and Mixed Tumor Service Department of Surgery and Gastroenterology Service Department of Medicine Memorial Sloan-Kettering Cancer Center New York, New York A congenital diaphragm is an extremely rare cause of duodenal obstruction in adult life and is usually not diagnosed before operation. Halka et al. ' were the first to report the endoscopic features of this deformity, describing a "cervix sign" characterized by a narrow nipple-shaped lumen with circumferential cuffing of the duodenal muco~a at the apex of the bulb giving the appearance of a cervical os. The correct pre-operative diagnosis was also made in the patient reported by Hudson 2 who noted that, in the 11 previously recorded cases, only 1 had been recognized before operation and that a perforated web or diaphragm is probably the only congenital form of intrinsic duodenal obstruction that can remain undiagnosed until adult life. An extensive literature review by Kreig 3 revealed that congenital diaphragms occurred in only 0.8% to 2.5% of all patients with duodenal obstruction or malformation and may be encountered throughout life. The oldest patient described was in the seventh decade, and 29% of patients did not develop symptoms until after 24 years of age. The following adult patient is reported to confirm the characteristic endoscopic and radiographic findings of this condition. The technique used for removal of the diaphragm suggests that subsequent patients might be managed entirely endoscopically with biopsy forceps and electrocautery. CASE REPORT A 56-year-old, obese, white, male physician was admitted in May 1979 with complaints of increasing upper abdominal girth, a weight gain of approximately 20 • Reprint requests: A. Turnbull. MD. Department of Surgery, Memorial Sloan-Kettering Cancer Center. 127S York Avenue. New York, New York 10021.
46
pounds, early satiety, and regurgitation relieved by induced vomiting, all of indefinite duration. His past history revealed no illness pertaining to the gastrointestinal tract; he had enjoyed good health and a generous caloric intake. Barium meal showed massive dilation of the entire stomach with megaduodenum to the level of the superior mesenteric vessels (Figure 1A). There was a large amount of partially digested meat and vegetable matter congealed in his stomach which required several days of nasogastric lavage and drainage before endoscopy could be attempted. During this period, celiac and superior mesenteric arteriography and computerized axial tomography revealed no tumor in the proximal pancreas. The stomach remained distended and patulous despite continuous drainage, and 2 experienced endoscopists were unable to traverse the pyloric canal with a gastroscope to examine the duodenum. Their difficulty was attributed to coiling of the 'scope in the very large antrum, preventing forward movement into the duodenum. After appropriate fluid and electrolyte balance was assured, surgical exploration .revealed a massively distended stomach with extremely thick walls, a very patulous pyloric canal, and a distended duodenum down to the level of the superior mesenteric vessels. Careful examination of the remainder of the abdomen did not demonstrate any other visible or palpable abnormalities. The hepatic flexure of the colon was mobilized, and no obvious extrinsic or intrinsic cause of duodenal compression was found after the entire course of the duodenum was exposed and examined. Because an intrinsic, possibly congenital abnormality was anticipated, the pyloric channel was incised into the proximal duodenum. Digital examination revealed an obstruction with a central orifice at the level of the superior mesenteric vessels which barely admitted the tip of the examining finger. A sterile rigid proctoscope was inserted through the duodenal opening and the diaphragm was visualized. External examination at the site of the obstruction revealed no fibrosis, scarring, or bands. To record these findings, a fiberoptic esophagoscope was inserted which revealed the "cervix sign" and an estimated aperture of 8 mm (Figure 18). The excellent exposure provided by the rigid proctoscope suggested transluminal removal which was done with forward-biting biopsy forceps. Generous pieces of the diaphragm were taken from its 4 quadrants under direct visualization, while an index finger guided the forceps from outside to ensure that only the diaphragm itself was taken. Frozen and subsequent paraffin sections revealed normal duodenal mucosa with fibrosis, glandular atypia, ectasia, and chronic inflammation. After these 4 GASTROINTESTINAL ENDOSCOPY