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Appendicitis as a complication of colonoscopy
ter duodenoscopic sphincterotomy for stones in patients with prior cholecystectomy.Gastroenterology 1990;98:1008-12. 8. Smith AC, Schapiro RH, Kelsey PB, Warshaw AL. Successful treatment of nonhealing biliary-cutaneous fistulas with biliary stents. Gastroenterology 1986;90:764-9. 9. Gholson CF, Burton F. Closure of controlled biliary fistula complicating partial cholecystectomy with endoscopic bfliary stenting. Am J Gastroenterol 1992;87:248-51.
Appendicitis as a complication of colonoscopy Ronald Vender, Jessie Larson, Juan Garcia, Mark Topazian, Paul Ephraim,
MD MD MD MD MD
Diagnostic colonoscopy is a relatively low-risk procedure, although the risk is slightly increased with t h e r a p e u t i c interventions. P e r f o r a t i o n and bleeding are the most c o m m o n serious complications related to this procedure. We r e p o r t three cases of appendicitis t h a t developed after successful, uncomplicated colonoscopies during which no biopsy or p o l y p e c t o m y was performed.
CASE REPORTS Case 1 A 44-year-old woman with a history of adenomatous polyps presented for colonoscopy. Her last colonoscopy with polypectomy had been done 2 years previously without complications. She had chronic abdominal cramping associated with constipation, attributed to irritable bowel syndrome. Her family history was notable for a grandfather and paternal cousin with carcinoma of the colon. She took no medications. The patient underwent colonoscopy to the cecum, which was normal. Although some redundancy of the colon was noted, the colonoscope was advanced without great difficulty. The preparation was excellent, requiring no cecal washing. The colonoscope was not impacted against the appendiceal orifice. No biopsy or polypectomy was done. The patient did well after the procedure and was discharged home. Later that evening, she called to report right lower quadrant pain, fever, chills, and nausea. In the emergency department she was in mild distress. Her blood pressure was
From the Yale University School of Medicine, and the Hospital of Saint Raphael, New Haven, Connecticut. Reprint requests: Ronald Vender, MD, Hospital of St. Raphael, Dept. of Gastroenterology, 1450 Chapel Street, New Haven, CT 06511. 0016-5107/95/4105-051453.00 + 0 GASTROINTESTINAL ENDOSCOPY Copyright | 1995 by the American Society for Gastrointestinal Endoscopy
37/4/57404 514
GASTROINTESTINAL ENDOSCOPY
10. Sherman S, Ruffolo TA, Hawes RH, Lehman GA. Complications of endoscopic sphincterotomy: a prospective series with emphasis on the increased risk associated with sphincter of Oddi dysfunction and nondilated bile ducts. Gastroenterology 1991; 101:1068-75.
100/70 mm Hg, heart rate 100, and temperature 101.4 ~ Her abdomen was mildly distended with rebound tenderness in the right lower quadrant. Chest and abdominal radiography findings were normal. The white blood cell count was 16,500 and hematocrit was 31 g/dL. CT of the abdomen showed stranding posterior to the right colon with extension to the right psoas muscle, where small hypodense collections were noted. Cecal wall thickening was noted associated with an amorphous collection of dense contrast between the right iliac vessels and urinary bladder. It was unclear whether this collection was extraintestinal or within the thick-walled cecum. The patient underwent exploratory laparotomy. Acute appendicitis was found, and an appendectomy was performed. Postoperatively, she had an uneventful recovery. She continues to do well when seen in follow-up.
Case 2 A 55-year-old man underwent a screening colonoscopy because of a strong family history of carcinoma of the colon. He felt well before the colonoscopy and had no bowel symptoms. Colonoscopy to the cecum was uneventful, and no abnormalities were noted. Adherent stool was washed off the cecal wall during the procedure, but the colonoscope was not impacted against the appendiceal orifice. No biopsy or polypectomy was performed. The patient felt well after the colonoscopy and was discharged home. Two days later the patient reported right lower quadrant abdominal pain, which had begun on the evening of colonoscopy. Initially the discomfort subsided but recurred on the second evening. His pain then steadily worsened, and he presented to the emergency department with fever and chills. On examination his temperature was 100.8 ~ and heart rate was 80. His abdomen was soft with right lower quadrant tenderness but without guarding, distention, or rebound. Bowel sounds were normal. His white blood cell count was 15,200 with a hematocrit of 47 g/dL. Results of tests for liver function were normal except for an ALT of 49 IU/L (normal range, 15 to 35). CT of the abdomen showed stranding around the pole of the cecum with a fluid-filled, thick-walled appendix. The patient was evaluated by a surgeon. In view of the lack of acute peritoneal signs, medical treatment with intravenous antibiotics (ampicillin, gentamycin, metronidazole) and fluids was suggested. Within 24 hours the patient became afebrile with less pain and tenderness. By his third hospital day, the patient was able to eat and shortly thereafter was discharged on ampicillin/clavulanate potassium and metronidazole. He has since done well. VOLUME 41, NO. 5, 1995
Case 3
A 57-year-old woman with a 2-month history of diarrhea was referred for further evaluation of her altered bowel habits. She had a family history of carcinoma of the colon. A colonoscopy to the cecum was performed without any difficulties. The bowel preparation was excellent and good visualization of the cecal landmarks was obtained. The scope was not impacted against the cecal pole. No biopsy or polypectomy was performed. Diverticulosis of the sigmoid colon and internal hemorrhoids were seen. After the examination, bilateral lower quadrant pain developed, which persisted throughout the night. The patient's pain intensified, with radiation to the right lower quadrant. She presented to the emergency department with worsening abdominal pain and fever. Her past history included hypertension, migraine headaches, and a tubal ligation. Her medications were nifedipine and propranolol. Physical examination revealed a temperature of 101~ The abdomen was distended with right lower quadrant tenderness, guarding, and localized peritoneal signs. Initial white blood cell count was 16,700 and hematocrit was 39 g/dL. Chest x-ray films revealed no free air, and abdominal x-ray films showed stool in the right colon but no other abnormalities. During the next several hours, the patient's peritoneal signs worsened and the white blood cell count rose. At laparotomy a perforated, gangrenous appendix was seen. Histologic examination revealed acute suppurative appendicitis with the presence of a fecalith in the appendicular lumen. The patient has done well since surgery. DISCUSSION
Colonoscopy is a relatively safe procedure with known associated risks. Major complications include perforation, bleeding, and postpolypectomy syndrome. 15 Other complications include toxic megacolon, development of hepatic portal venous gas, submucosal dissection caused by insuffiated air, serosal tears, intra-abdominal hematoma or abscess formation, colonic obstruction, snare entrapment, splenic rupture, pneumomediastinum, pneumothorax, incarcerated hernia, transient bacteremia, ileus, diverticulitis, and appendicitis. 1-9 Appendicitis has been described in two prior reports. In 1988, Houghton and Aston 6 reported on a 35-year-old man who underwent uncomplicated diagnostic colonoscopy for evaluation of lower abdominal pain and constipation; acute appendicitis developed 8 hours later. In the report by Brandt and Naess, 7 a 65-year-old woman with polycystic kidney disease required a colonoscopy for pretransplant evaluation after initial barium enema radiography revealed a polyp in the sigmoid colon. Colonoscopy with polypectomy was successful, and no immediate complications occurred. Several days after the procedure, abdominal pain and fever developed and a diagnosis of appendicitis was made. Our experience suggests that appendicitis after VOLUME 41, NO. 5, 1995
colonoscopy may be more than a chance occurrence. This cluster of cases, each performed by a different endoscopist, might have gone unrecognized had they not been presented at a morbidity and mortality conference. During the past 2 years, these three cases occurred at two institutions where a total of approximately 8000 colonoscopies were performed. Whether this represents an unusual cluster of cases or a previously underreported complication remains to be seen. The mechanisms by which colonoscopy might induce appendicitis are unknown, but they could be similar to those suggested for diverticulitis complicating colonoscopy.9, 10 These include (1) pre-existing subclinical disease of the appendix, (2) barotrauma resulting from overinsuffiation, (3) introduction of a fecalith into the appendix, causing obstruction or inflammation, and (4) direct intubation of the appendiceal lumen. It is also possible for residual glutaraldehyde in the endoscope channel to be introduced into the appendix during washing of the cecum, causing inflammation. In all cases it was well documented that no overinflation of the bowel or impaction against the appendiceal orifice occurred. In only one case was cecal washing done. We do not think that the endoscopic technique contributed to the development of appendicitis in these cases. The pathologic examination showed evidence of inflammation in the two cases. A fecalith was embedded in the wall of the appendix in the case of appendiceal perforation. Whether the presence of the fecalith was a contributory factor or a coincidental finding is difficult to determine. Our patients initially felt well after colonoscopy and were discharged home. In all cases, symptoms developed on the evening of the procedure. The two patients who returned immediately for evaluation had focal peritoneal findings on physical examination and underwent laparotomy, which confirmed the diagnosis of acute appendicitis. The third patient did not report his symptoms until 2 days after colonoscopy, and these were milder than in the other cases. CT of the abdomen demonstrated changes consistent with appendicitis, without perforation or abscess. Because of the absence of peritoneal findings on examination, he was treated medically with intravenous antibiotics and rapidly improved. Appendicitis is a rare but important complication of colonoscopy. Prompt recognition of this complication should lead to early and effective treatment. Appendicitis should be treated according to the patient's clinical course regardless of the cause.
REFERENCES 1. WayeJ, LewisB, VessayanS. Colonoscopy:a prospectivereport of complications. J Clin Gastroenterol 1992;15:347-51. 2. MacraeF, Tan K, WilliamsC. Towardssafercolonoscopy:a reGASTROINTESTINAL ENDOSCOPY 5 1 5
3. 4. 5. 6.
port on the complications of 5000 diagnostic or therapeutic colonoscopies. Gut 1983;24:376-83. Wu T. Occult injuries during colonoscopy. Measurement of the forces required to injure the colon and report of the cases. Gastrointest Endosc 1978;24:236-8. Ghazi A, Grossman M. Complications of colonoscopy and polypectomy. Surg Clin North Am 1982;62:889-96. Smith L. Fiberoptic colonoscopy: complications of colonoscopy and polypectomy. Dis Colon Rectum 1976;19:407-12. Houghton A, Aston N. Appendicitis complicating colonoscopy [Letter]. Gastrointest Endosc 1988;34:489.
Endoscopic features of long-standing primary intestinal lymphangiectasia Howard A. Salomons, Phillip Kramer, Sigfus Nikulasson, Paul C. Schroy,
MD MD MD MD
P r i m a r y intestinal l y m p h a n g i e c t a s i a (PIL) is a rare congenital disorder characterized by dilated a n d tort u o u s intestinal lacteals resulting f r o m i m p a i r e d lymphatic drainage. Despite its name, the condition is often generalized a n d can affect l y m p h a t i c drainage anywhere in the body. P r o m i n e n t clinical features include protein-losing e n t e r o p a t h y , l y m p h o c y t o p e n i a , steatorrhea, p e r i p h e r a l edema, and chylous effusions as originally described by W a l d m a n n et al. 1 T h e natural history of this rare disorder is not well k n o w n as only a few cases have been r e p o r t e d in the literature. We p r e s e n t herein the l o n g - t e r m follow-up of a p a t i e n t with P I L a n d the associated endoscopic, radiographic, a n d histologic features of the disorder.
CASE REPORT A 40-year-old woman initially presented to our institution at age 14 with progressive edema of the lower extremities, diarrhea, and chylothorax. Results of laboratory studies indicated lymphocytopenia, hypogammagtobulinemia, and hypoalbuminemia. An upper gastrointestinal series with small bowel follow-through (UGI/SBFT) revealed coarse mucosal folds and dilatation of the duodenum and jejunum. Small bowel biopsy with the Crosby capsule demonstrated markedly dilated and distended lymphatics within the villi and marked edema of the lamina propria, consistent with intestinal lymphangiectasia. She was subsequently placed on a low-fat diet supplemented with medium-chain triglycerides (MCT). All symptoms gradually resolved except for occasional bouts of lower extremity edema.
From the Departments of Gastroenterology and Pathology, Boston University Medical Center, Boston, Massachusetts. Reprint requests: Paul C. Schroy, MD, Boston University Medical Center, University Hospital, Division of Gastroenterology, E201, 88 East Newton St. Boston, MA, 02118 0016-5107/95/4105-051653.00 + 0 GASTROINTESTINAL ENDOSCOPY Copyright | 1995 by the American Society for Gastrointestinal Endoscopy 37/4/58483
516
GASTROINTESTINAL ENDOSCOPY
7. Brandt T, Naess A. Acute appendicitis following endoscopic polypectomy. Endoscopy 1989;21:44. 8. Barnett T, McGeehin W, Chen C, Brennan E. Acute tension pneumoperitoneum following colonoscopy. Gastrointest Endosc 1992;38:99-100. 9. Razzak I, Millan J, Schuster M. Pneumatic ileal perforation: an unusual complication of colonoscopy. Gastroenterology t976; 70:268-71. 10. Freeman S, McNally P. Diverticulitis. In: Chohan N, Wong R, eds. Med Clin North Am 1993;77:1149-67.
The patient remained well until age 40, when she presented with a 6-month history of fevers to 102 ~F, chills, night sweats, shortness of breath, progressive lower extremity edema, and intermittent diarrhea described as white in appearance. Physical examination revealed an afebrile woman in no distress. She had decreased breath sounds with dullness to percussion at the right lung base, 2/6 systolic ejection murmur, mild right upper quadrant tenderness without hepatosplenomegaly, and 3+ pitting edema of both lower extremities extending proximally to the midthighs. Laboratory findings included white blood cell count 7000/mm 3 with no lymphocytes, total protein 4.0 g/dL, albumin 1.4 g/dL, gamma globulin 0.8 g/dL (normal, 0.6 to 1.6 g/dL), erythrocyte sedimentation rate 62 ram/h, vitamin A 15 #g/dL (norreal, 30 to 95 #g/dL), 25-hydroxycholecalciferol 6 ng/mL (normal, 10 to 55 ng/mL). Blood, urine, and sputum bacterial cultures were negative, as were tests for antinuclear antibody and rheumatoid factor. Chest films demonstrated a small right pleural effusion, which was subsequently tapped and found to be chylous in nature with a triglyceride level of 364 mg/dL. CT of the abdomen was remarkable for mediastinal, retrocrural, and retroperitoneal lymphadenopathy; thickening of the proximal small bowel in the region of the jejunum, and a 1.5-cm cyst in the lower part of the spleen. A UGI/SBFT demonstrated multiple round filling defects within the mucosa of the proximal and middle segments of the small bowel, particularly in the region of the duodenal sweep; the terminal ileum appeared normal (Fig. 1). Because of the findings on UGI/SBFT, an enteroscopy was performed with the Olympus PCF-100 pediatric video colonoscope (Olympus America Inc., Lake Success, N.Y.) to the proximal jejunum. Multiple submucosal nodules and well-circumscribed xanthomatous plaques of varying sizes were noted throughout the duodenum and jejunum (Fig. 2A-D). The mucosa was remarkable for a prominent whitetipped villous pattern. A 3-cm multilobulated cystic mass observed within the duodenal sweep had an erythematous and friable overlying mucosa (Fig. 2). Biopsy of this cystic mass resulted in an exudation of white, chylous material. Histologic examination revealed dilatation of lacteals within the villi, consistent with the diagnosis of intestinal lymphangiectasia; no evidence of lymphoma was noted (Fig. 3). The patient was again placed on a low-fat, MCT-supptemented diet. She was also started on vitamin supplements because of the decreased levels of vitamins A and D. Her condition rapidly improved, and at a 3-month follow-up visit she was essentially asymptomatic with significantly less peripheral edema, a serum albumin of 2.9 g/dL, and normal levels of vitamins A and D.
VOLUME 41, NO. 5, 1995
Appendicitis as a complication of colonoscopy Ronald Vender, Jessie Larson, Juan Garcia, Mark Topazian, Paul Ephraim,
MD MD MD MD MD
Diagnostic colonoscopy is a relatively low-risk procedure, although the risk is slightly increased with t h e r a p e u t i c interventions. P e r f o r a t i o n and bleeding are the most c o m m o n serious complications related to this procedure. We r e p o r t three cases of appendicitis t h a t developed after successful, uncomplicated colonoscopies during which no biopsy or p o l y p e c t o m y was performed.
CASE REPORTS Case 1 A 44-year-old woman with a history of adenomatous polyps presented for colonoscopy. Her last colonoscopy with polypectomy had been done 2 years previously without complications. She had chronic abdominal cramping associated with constipation, attributed to irritable bowel syndrome. Her family history was notable for a grandfather and paternal cousin with carcinoma of the colon. She took no medications. The patient underwent colonoscopy to the cecum, which was normal. Although some redundancy of the colon was noted, the colonoscope was advanced without great difficulty. The preparation was excellent, requiring no cecal washing. The colonoscope was not impacted against the appendiceal orifice. No biopsy or polypectomy was done. The patient did well after the procedure and was discharged home. Later that evening, she called to report right lower quadrant pain, fever, chills, and nausea. In the emergency department she was in mild distress. Her blood pressure was
From the Yale University School of Medicine, and the Hospital of Saint Raphael, New Haven, Connecticut. Reprint requests: Ronald Vender, MD, Hospital of St. Raphael, Dept. of Gastroenterology, 1450 Chapel Street, New Haven, CT 06511. 0016-5107/95/4105-051453.00 + 0 GASTROINTESTINAL ENDOSCOPY Copyright | 1995 by the American Society for Gastrointestinal Endoscopy
37/4/57404 514
GASTROINTESTINAL ENDOSCOPY
10. Sherman S, Ruffolo TA, Hawes RH, Lehman GA. Complications of endoscopic sphincterotomy: a prospective series with emphasis on the increased risk associated with sphincter of Oddi dysfunction and nondilated bile ducts. Gastroenterology 1991; 101:1068-75.
100/70 mm Hg, heart rate 100, and temperature 101.4 ~ Her abdomen was mildly distended with rebound tenderness in the right lower quadrant. Chest and abdominal radiography findings were normal. The white blood cell count was 16,500 and hematocrit was 31 g/dL. CT of the abdomen showed stranding posterior to the right colon with extension to the right psoas muscle, where small hypodense collections were noted. Cecal wall thickening was noted associated with an amorphous collection of dense contrast between the right iliac vessels and urinary bladder. It was unclear whether this collection was extraintestinal or within the thick-walled cecum. The patient underwent exploratory laparotomy. Acute appendicitis was found, and an appendectomy was performed. Postoperatively, she had an uneventful recovery. She continues to do well when seen in follow-up.
Case 2 A 55-year-old man underwent a screening colonoscopy because of a strong family history of carcinoma of the colon. He felt well before the colonoscopy and had no bowel symptoms. Colonoscopy to the cecum was uneventful, and no abnormalities were noted. Adherent stool was washed off the cecal wall during the procedure, but the colonoscope was not impacted against the appendiceal orifice. No biopsy or polypectomy was performed. The patient felt well after the colonoscopy and was discharged home. Two days later the patient reported right lower quadrant abdominal pain, which had begun on the evening of colonoscopy. Initially the discomfort subsided but recurred on the second evening. His pain then steadily worsened, and he presented to the emergency department with fever and chills. On examination his temperature was 100.8 ~ and heart rate was 80. His abdomen was soft with right lower quadrant tenderness but without guarding, distention, or rebound. Bowel sounds were normal. His white blood cell count was 15,200 with a hematocrit of 47 g/dL. Results of tests for liver function were normal except for an ALT of 49 IU/L (normal range, 15 to 35). CT of the abdomen showed stranding around the pole of the cecum with a fluid-filled, thick-walled appendix. The patient was evaluated by a surgeon. In view of the lack of acute peritoneal signs, medical treatment with intravenous antibiotics (ampicillin, gentamycin, metronidazole) and fluids was suggested. Within 24 hours the patient became afebrile with less pain and tenderness. By his third hospital day, the patient was able to eat and shortly thereafter was discharged on ampicillin/clavulanate potassium and metronidazole. He has since done well. VOLUME 41, NO. 5, 1995
Case 3
A 57-year-old woman with a 2-month history of diarrhea was referred for further evaluation of her altered bowel habits. She had a family history of carcinoma of the colon. A colonoscopy to the cecum was performed without any difficulties. The bowel preparation was excellent and good visualization of the cecal landmarks was obtained. The scope was not impacted against the cecal pole. No biopsy or polypectomy was performed. Diverticulosis of the sigmoid colon and internal hemorrhoids were seen. After the examination, bilateral lower quadrant pain developed, which persisted throughout the night. The patient's pain intensified, with radiation to the right lower quadrant. She presented to the emergency department with worsening abdominal pain and fever. Her past history included hypertension, migraine headaches, and a tubal ligation. Her medications were nifedipine and propranolol. Physical examination revealed a temperature of 101~ The abdomen was distended with right lower quadrant tenderness, guarding, and localized peritoneal signs. Initial white blood cell count was 16,700 and hematocrit was 39 g/dL. Chest x-ray films revealed no free air, and abdominal x-ray films showed stool in the right colon but no other abnormalities. During the next several hours, the patient's peritoneal signs worsened and the white blood cell count rose. At laparotomy a perforated, gangrenous appendix was seen. Histologic examination revealed acute suppurative appendicitis with the presence of a fecalith in the appendicular lumen. The patient has done well since surgery. DISCUSSION
Colonoscopy is a relatively safe procedure with known associated risks. Major complications include perforation, bleeding, and postpolypectomy syndrome. 15 Other complications include toxic megacolon, development of hepatic portal venous gas, submucosal dissection caused by insuffiated air, serosal tears, intra-abdominal hematoma or abscess formation, colonic obstruction, snare entrapment, splenic rupture, pneumomediastinum, pneumothorax, incarcerated hernia, transient bacteremia, ileus, diverticulitis, and appendicitis. 1-9 Appendicitis has been described in two prior reports. In 1988, Houghton and Aston 6 reported on a 35-year-old man who underwent uncomplicated diagnostic colonoscopy for evaluation of lower abdominal pain and constipation; acute appendicitis developed 8 hours later. In the report by Brandt and Naess, 7 a 65-year-old woman with polycystic kidney disease required a colonoscopy for pretransplant evaluation after initial barium enema radiography revealed a polyp in the sigmoid colon. Colonoscopy with polypectomy was successful, and no immediate complications occurred. Several days after the procedure, abdominal pain and fever developed and a diagnosis of appendicitis was made. Our experience suggests that appendicitis after VOLUME 41, NO. 5, 1995
colonoscopy may be more than a chance occurrence. This cluster of cases, each performed by a different endoscopist, might have gone unrecognized had they not been presented at a morbidity and mortality conference. During the past 2 years, these three cases occurred at two institutions where a total of approximately 8000 colonoscopies were performed. Whether this represents an unusual cluster of cases or a previously underreported complication remains to be seen. The mechanisms by which colonoscopy might induce appendicitis are unknown, but they could be similar to those suggested for diverticulitis complicating colonoscopy.9, 10 These include (1) pre-existing subclinical disease of the appendix, (2) barotrauma resulting from overinsuffiation, (3) introduction of a fecalith into the appendix, causing obstruction or inflammation, and (4) direct intubation of the appendiceal lumen. It is also possible for residual glutaraldehyde in the endoscope channel to be introduced into the appendix during washing of the cecum, causing inflammation. In all cases it was well documented that no overinflation of the bowel or impaction against the appendiceal orifice occurred. In only one case was cecal washing done. We do not think that the endoscopic technique contributed to the development of appendicitis in these cases. The pathologic examination showed evidence of inflammation in the two cases. A fecalith was embedded in the wall of the appendix in the case of appendiceal perforation. Whether the presence of the fecalith was a contributory factor or a coincidental finding is difficult to determine. Our patients initially felt well after colonoscopy and were discharged home. In all cases, symptoms developed on the evening of the procedure. The two patients who returned immediately for evaluation had focal peritoneal findings on physical examination and underwent laparotomy, which confirmed the diagnosis of acute appendicitis. The third patient did not report his symptoms until 2 days after colonoscopy, and these were milder than in the other cases. CT of the abdomen demonstrated changes consistent with appendicitis, without perforation or abscess. Because of the absence of peritoneal findings on examination, he was treated medically with intravenous antibiotics and rapidly improved. Appendicitis is a rare but important complication of colonoscopy. Prompt recognition of this complication should lead to early and effective treatment. Appendicitis should be treated according to the patient's clinical course regardless of the cause.
REFERENCES 1. WayeJ, LewisB, VessayanS. Colonoscopy:a prospectivereport of complications. J Clin Gastroenterol 1992;15:347-51. 2. MacraeF, Tan K, WilliamsC. Towardssafercolonoscopy:a reGASTROINTESTINAL ENDOSCOPY 5 1 5
3. 4. 5. 6.
port on the complications of 5000 diagnostic or therapeutic colonoscopies. Gut 1983;24:376-83. Wu T. Occult injuries during colonoscopy. Measurement of the forces required to injure the colon and report of the cases. Gastrointest Endosc 1978;24:236-8. Ghazi A, Grossman M. Complications of colonoscopy and polypectomy. Surg Clin North Am 1982;62:889-96. Smith L. Fiberoptic colonoscopy: complications of colonoscopy and polypectomy. Dis Colon Rectum 1976;19:407-12. Houghton A, Aston N. Appendicitis complicating colonoscopy [Letter]. Gastrointest Endosc 1988;34:489.
Endoscopic features of long-standing primary intestinal lymphangiectasia Howard A. Salomons, Phillip Kramer, Sigfus Nikulasson, Paul C. Schroy,
MD MD MD MD
P r i m a r y intestinal l y m p h a n g i e c t a s i a (PIL) is a rare congenital disorder characterized by dilated a n d tort u o u s intestinal lacteals resulting f r o m i m p a i r e d lymphatic drainage. Despite its name, the condition is often generalized a n d can affect l y m p h a t i c drainage anywhere in the body. P r o m i n e n t clinical features include protein-losing e n t e r o p a t h y , l y m p h o c y t o p e n i a , steatorrhea, p e r i p h e r a l edema, and chylous effusions as originally described by W a l d m a n n et al. 1 T h e natural history of this rare disorder is not well k n o w n as only a few cases have been r e p o r t e d in the literature. We p r e s e n t herein the l o n g - t e r m follow-up of a p a t i e n t with P I L a n d the associated endoscopic, radiographic, a n d histologic features of the disorder.
CASE REPORT A 40-year-old woman initially presented to our institution at age 14 with progressive edema of the lower extremities, diarrhea, and chylothorax. Results of laboratory studies indicated lymphocytopenia, hypogammagtobulinemia, and hypoalbuminemia. An upper gastrointestinal series with small bowel follow-through (UGI/SBFT) revealed coarse mucosal folds and dilatation of the duodenum and jejunum. Small bowel biopsy with the Crosby capsule demonstrated markedly dilated and distended lymphatics within the villi and marked edema of the lamina propria, consistent with intestinal lymphangiectasia. She was subsequently placed on a low-fat diet supplemented with medium-chain triglycerides (MCT). All symptoms gradually resolved except for occasional bouts of lower extremity edema.
From the Departments of Gastroenterology and Pathology, Boston University Medical Center, Boston, Massachusetts. Reprint requests: Paul C. Schroy, MD, Boston University Medical Center, University Hospital, Division of Gastroenterology, E201, 88 East Newton St. Boston, MA, 02118 0016-5107/95/4105-051653.00 + 0 GASTROINTESTINAL ENDOSCOPY Copyright | 1995 by the American Society for Gastrointestinal Endoscopy 37/4/58483
516
GASTROINTESTINAL ENDOSCOPY
7. Brandt T, Naess A. Acute appendicitis following endoscopic polypectomy. Endoscopy 1989;21:44. 8. Barnett T, McGeehin W, Chen C, Brennan E. Acute tension pneumoperitoneum following colonoscopy. Gastrointest Endosc 1992;38:99-100. 9. Razzak I, Millan J, Schuster M. Pneumatic ileal perforation: an unusual complication of colonoscopy. Gastroenterology t976; 70:268-71. 10. Freeman S, McNally P. Diverticulitis. In: Chohan N, Wong R, eds. Med Clin North Am 1993;77:1149-67.
The patient remained well until age 40, when she presented with a 6-month history of fevers to 102 ~F, chills, night sweats, shortness of breath, progressive lower extremity edema, and intermittent diarrhea described as white in appearance. Physical examination revealed an afebrile woman in no distress. She had decreased breath sounds with dullness to percussion at the right lung base, 2/6 systolic ejection murmur, mild right upper quadrant tenderness without hepatosplenomegaly, and 3+ pitting edema of both lower extremities extending proximally to the midthighs. Laboratory findings included white blood cell count 7000/mm 3 with no lymphocytes, total protein 4.0 g/dL, albumin 1.4 g/dL, gamma globulin 0.8 g/dL (normal, 0.6 to 1.6 g/dL), erythrocyte sedimentation rate 62 ram/h, vitamin A 15 #g/dL (norreal, 30 to 95 #g/dL), 25-hydroxycholecalciferol 6 ng/mL (normal, 10 to 55 ng/mL). Blood, urine, and sputum bacterial cultures were negative, as were tests for antinuclear antibody and rheumatoid factor. Chest films demonstrated a small right pleural effusion, which was subsequently tapped and found to be chylous in nature with a triglyceride level of 364 mg/dL. CT of the abdomen was remarkable for mediastinal, retrocrural, and retroperitoneal lymphadenopathy; thickening of the proximal small bowel in the region of the jejunum, and a 1.5-cm cyst in the lower part of the spleen. A UGI/SBFT demonstrated multiple round filling defects within the mucosa of the proximal and middle segments of the small bowel, particularly in the region of the duodenal sweep; the terminal ileum appeared normal (Fig. 1). Because of the findings on UGI/SBFT, an enteroscopy was performed with the Olympus PCF-100 pediatric video colonoscope (Olympus America Inc., Lake Success, N.Y.) to the proximal jejunum. Multiple submucosal nodules and well-circumscribed xanthomatous plaques of varying sizes were noted throughout the duodenum and jejunum (Fig. 2A-D). The mucosa was remarkable for a prominent whitetipped villous pattern. A 3-cm multilobulated cystic mass observed within the duodenal sweep had an erythematous and friable overlying mucosa (Fig. 2). Biopsy of this cystic mass resulted in an exudation of white, chylous material. Histologic examination revealed dilatation of lacteals within the villi, consistent with the diagnosis of intestinal lymphangiectasia; no evidence of lymphoma was noted (Fig. 3). The patient was again placed on a low-fat, MCT-supptemented diet. She was also started on vitamin supplements because of the decreased levels of vitamins A and D. Her condition rapidly improved, and at a 3-month follow-up visit she was essentially asymptomatic with significantly less peripheral edema, a serum albumin of 2.9 g/dL, and normal levels of vitamins A and D.
VOLUME 41, NO. 5, 1995