- Email: [email protected]
Intestinal obstruction due to protein casts
I N T E S T I N A L O B S T R U C T I O N DUE TO P R O T E I N CASTS LEO ,J. GEPPERT, COLONEL, M C , U S A FORT SAM HOUSTON, TEXAS
X C E P T in veterinary medicine, where it receives first priority, abnormal contents of the intestinal tract are rarely considered in the differentia] diagnosis of intestinal obstruction. This is inexplicable since fecal impaction is probably the most common and the oldest recognized cause of obstruction. Meeonium ileus is a well-reeog-nized manifestation of cystic fibrosis of the pancreas. A recent review which includes the postoperative proguosis of this type of obstruction is available. ~ Years ago Falls and Jaffe 2 pointed out that the anorectal plug or "meconiunl w o r m " is frequently the sole cause of intestinal obstruction in newborn infants. Since all newborn infants have (or have had) such a plug, a 12-hour period of observation following its removal is necessary before surgical exploration for a more serious disease is undertaken (provided that there is no evidence of compromised mesenteric circulation). Mechanical obstruction should still be suspected. It has been my experience that the plug is less likely to be expressed by newborn infants with atresia of the intestine (see Fig. 1, A). Abnormalities in tim meconium of the fetus and newborn infant were thoroughly covered in a recent paper by Emery, ~ and the clinical aspects outlined in cases reported by Zachary. 4 Obstruction and perforation may result from intcstinal fecal casts as well
as from classical meeoniulu ileus ~ (see Fig. 1, B). Levy, 6 in 1951, first reported a case of late fecal obstruction associated with cystic fibrosis of the pancreas in a 7-month-old infant. He emphasized the altered physical state of fecal contents which resulted in puttylike feces responsible for intestinal obstruction beyond the newborn period. More recent case reports and discussions of " l a t e meeonium ileus" are provided by Fisher, ~ Nixon, s Birse, 9 and Andersen. 1~ Shwachman u emphasized that the obstructing material in the intestinal lumen may be either puttylike or hard and rocklike. I have been impressed with the combination of impactive obstruction and paradoxical diarrhea in these patients. Photographs of such specimens were taken but were not included because they cannot be distinguished from photographs of molding putty, new or dried out, and are familiar to all. (The father of one patient had often picked up pieces of tiffs material from the floor and made models with it, thinking it was molding clay carelessly left around by his child.) The following ease history describes the results of another type of intraluminal cast. This 2-month-old infant with cystic fibrosis of the pancreas suddenly had his entire intestine obstructed by precipitated milk protein (see Fig. 1, C).
From the Pediatric Service. Department of Medicine, Brooke Army Hospit~tl, Brooke ~ r m y Medical Center,
This patient was one of twins born to a 28-year-old mother. There were
E
S18
CASE REPORT
814
T H E J O U R N A L OF P E D I A T R I C S
no siblings, and the family history was noncontributory. The twins were fed Bremil (Borden's) and had no particular difficulty d u r i n g the first 2 months of life. However, because of too frequent stools, they were suddenly changed from Bremil to 13 ounces of evaporated milk and 13 ounces of water, with no added sugar, 48 hours before admission. One twin tolerated this formula, but the other began
loops of bowel, but no free air in the peritoneal cavity. White blood cell count was 7,700; differential, normal; platelet count, 64,000 per cubic millimeter; blood u r e a nitrogen, 15 rag. per cent; chloride, 98.4 mEq. per liter; carbon dioxide 15 mEq. per liter. The diagnosis of intestinal obstruction was made, and an exploratory operation was performed. The ab-
Fig. 1.
vomiting, became listless and obviously uncomfortable, H e had intermittent intestinal discomfort. He h a d no stools after the formula change. Five enemas produced only mucus. The patient was admitted to Walter Reed A r m y Hospital on Jan. 26, 1956. His temperature was 101.4 ~ F. He was well developed and well nourished but was quite listless. The abdomen was distended and difficult to examine, but dilated loops of bowel were evident. Occasional bowel sounds were heard. The reetosigmoid was empty. X-ray examination showed dilated
domen was distended. When opened through a right paramedian incision, the intestines p r o t r u d e d immediately through the incision. The entire bowel appeared viable, and there was no obvious extraluminal cause for the obstruction. The proximal three fourths of the small intestine was greatly dilated. The distal third was contracted and filled with an opalescent material which looked and felt much like the white of a hard boiled egg. The colon was empty and collapsed. The operator attempted to milk the abnormal fecal material into the cecum
GEPPERT:
INTESTINAL OBSTRUCTION DUE TO PROTEIN CASTS
with only partial success. Because of the infant's general condition, f u r t h e r attempts were deemed contraindicated. The abdomen was therefore closed. Postoperative duodenal drainage revealed only traces of tryptic activity and 0.6 per cent unit of lipase. The child passed pencil-sized fragments of what appeared to be coagulated protein. Microscopic appearance was reported as " a m o r p h o u s material with no structural p a t t e r n . " F a t stains showed only a small amount of fat. Chemical analysis revealed that this material contained 65.8 rag. of nitrogen per gram (427.7 rag. of protein per gram), 66 rag. of fat per gram, 15.5 rag. of calcium per gram, 0.15 mEq. of sodium per gram, and .065 mEq. of potassium per gram ( d r y weight). The low potassium compared TABLE I.
SWEAT TESTS
TWIN A .. TWIN B AGE ~ CHLOCIILO(MONTHS) SODIUM RINE SODIUM RINE 61.0 90 88 72 92.3 109 150 180 133.0 127 178 172 Duodenal D~'ainage No trypsin No trypsin
with the sodium content suggests that this material at I1o time had the potassium-sodium ratio of 2:2 which is characteristic of living tissue. The protein composition was similar to that of evaporated milk. The child remained obstructed in spite of the instillation of pancreatic granules through a duodenal tube. A reopening of the laparotomy was therefore performed. The abnormal intestinal contents were forcibly removed from the terminal ileum through an ileostomy and a decompression ileostomy tube was left in place. Except for a transient episode of hypokalemia, the postoperative course was uneventful. Although there was no doubt that these children had pancreatic cystic fibrosis, both of them have done v e r y well with only minor gastrointestinal and respiratory symptoms. The diag-
8]5
nosis was, in fact, questioned and the sweat tests were repeated at another institution when the twins were 1 year old. The tests were again characteristic of this disorder. SUMMARY
1. A case of intestinal obstruction from a milk protein east of the entire intestine is reported. 2. Although this is believed to be the first such report, its actual ineidence in milder form in normal children as well as in infants with pancreatic fibrosis is open to speculation. 3. The latter part of this narrative also illustrates h o w asymptomatie cystic fibrosis of the pancreas may exist even when parents are aware of the diagnosis and are apprehensively searching for symptoms. REFERENCES ]. Shwachman, I-I., Pryles, C. V., and Gross, R. E.: Meconium Ileus: Clinical Study of 20 Surviving Patients, A. M. "A. J. Dis. Child. 91: 223, 1956. 2. Falls, F. H., and Jaffe, R. It.: Intestinal Obstruction in the Newborn Due to Mucus Plug, Am. J. Obst. & Gynec. 22: 409, 1931. 3. Emery, J. L.: Ab1{ormalities in Meconium of the Foetus and Newborn, Arch. Dis. Childhood 32: 17, 1957. 4. Zachary, R. ]3.: Meconium and Faecal Plugs in the Newborn, Arch. ])is. Childhood 32: 22, 1957. 5. Rogers, J. D., Baggenstoss, A. M., MusGrove, J. E., and Kennedy, R. L. J.: Intestinal Obstruction Associated With Fibrocystic Disease of the Pancreas, Minnesota Med. 34: 1075, 195l. 6. Levy, E.: A Case of Fibrocystic Disease of the Pancreas With Intestinal Obstruction, Arch. Dis. Childhood 26: 335, 1951. 7. Fisher, O. D.: Intestinal Obstruction as Late Complication of Fibrocystic Disease of Pa~icreas (Mueosis), Arch. ])is. Childhood 29: 262, 1954. 8. Nixon~ It. H.: Inspissation Obstruction of the Small Intestine in a Survivor From Meeonium Ileus, Great Ormond St. J. (no. 9), p. 43, 1955. 9. ]3irse, E. L.: Intestinal Obstruction as a Late Complication of Fibrocystic Disease of the Pancreas, Brit. M. J. 2: 286, 1956. 10. Andersen, D. H.: Cystic Fibrosis of the Pt~ncreas, J. Chron. Dis. 7: 58, 1958. 11. Shwachman, It.: Year ]3ook of Pediatrics, 1955-56, p. 203.
X C E P T in veterinary medicine, where it receives first priority, abnormal contents of the intestinal tract are rarely considered in the differentia] diagnosis of intestinal obstruction. This is inexplicable since fecal impaction is probably the most common and the oldest recognized cause of obstruction. Meeonium ileus is a well-reeog-nized manifestation of cystic fibrosis of the pancreas. A recent review which includes the postoperative proguosis of this type of obstruction is available. ~ Years ago Falls and Jaffe 2 pointed out that the anorectal plug or "meconiunl w o r m " is frequently the sole cause of intestinal obstruction in newborn infants. Since all newborn infants have (or have had) such a plug, a 12-hour period of observation following its removal is necessary before surgical exploration for a more serious disease is undertaken (provided that there is no evidence of compromised mesenteric circulation). Mechanical obstruction should still be suspected. It has been my experience that the plug is less likely to be expressed by newborn infants with atresia of the intestine (see Fig. 1, A). Abnormalities in tim meconium of the fetus and newborn infant were thoroughly covered in a recent paper by Emery, ~ and the clinical aspects outlined in cases reported by Zachary. 4 Obstruction and perforation may result from intcstinal fecal casts as well
as from classical meeoniulu ileus ~ (see Fig. 1, B). Levy, 6 in 1951, first reported a case of late fecal obstruction associated with cystic fibrosis of the pancreas in a 7-month-old infant. He emphasized the altered physical state of fecal contents which resulted in puttylike feces responsible for intestinal obstruction beyond the newborn period. More recent case reports and discussions of " l a t e meeonium ileus" are provided by Fisher, ~ Nixon, s Birse, 9 and Andersen. 1~ Shwachman u emphasized that the obstructing material in the intestinal lumen may be either puttylike or hard and rocklike. I have been impressed with the combination of impactive obstruction and paradoxical diarrhea in these patients. Photographs of such specimens were taken but were not included because they cannot be distinguished from photographs of molding putty, new or dried out, and are familiar to all. (The father of one patient had often picked up pieces of tiffs material from the floor and made models with it, thinking it was molding clay carelessly left around by his child.) The following ease history describes the results of another type of intraluminal cast. This 2-month-old infant with cystic fibrosis of the pancreas suddenly had his entire intestine obstructed by precipitated milk protein (see Fig. 1, C).
From the Pediatric Service. Department of Medicine, Brooke Army Hospit~tl, Brooke ~ r m y Medical Center,
This patient was one of twins born to a 28-year-old mother. There were
E
S18
CASE REPORT
814
T H E J O U R N A L OF P E D I A T R I C S
no siblings, and the family history was noncontributory. The twins were fed Bremil (Borden's) and had no particular difficulty d u r i n g the first 2 months of life. However, because of too frequent stools, they were suddenly changed from Bremil to 13 ounces of evaporated milk and 13 ounces of water, with no added sugar, 48 hours before admission. One twin tolerated this formula, but the other began
loops of bowel, but no free air in the peritoneal cavity. White blood cell count was 7,700; differential, normal; platelet count, 64,000 per cubic millimeter; blood u r e a nitrogen, 15 rag. per cent; chloride, 98.4 mEq. per liter; carbon dioxide 15 mEq. per liter. The diagnosis of intestinal obstruction was made, and an exploratory operation was performed. The ab-
Fig. 1.
vomiting, became listless and obviously uncomfortable, H e had intermittent intestinal discomfort. He h a d no stools after the formula change. Five enemas produced only mucus. The patient was admitted to Walter Reed A r m y Hospital on Jan. 26, 1956. His temperature was 101.4 ~ F. He was well developed and well nourished but was quite listless. The abdomen was distended and difficult to examine, but dilated loops of bowel were evident. Occasional bowel sounds were heard. The reetosigmoid was empty. X-ray examination showed dilated
domen was distended. When opened through a right paramedian incision, the intestines p r o t r u d e d immediately through the incision. The entire bowel appeared viable, and there was no obvious extraluminal cause for the obstruction. The proximal three fourths of the small intestine was greatly dilated. The distal third was contracted and filled with an opalescent material which looked and felt much like the white of a hard boiled egg. The colon was empty and collapsed. The operator attempted to milk the abnormal fecal material into the cecum
GEPPERT:
INTESTINAL OBSTRUCTION DUE TO PROTEIN CASTS
with only partial success. Because of the infant's general condition, f u r t h e r attempts were deemed contraindicated. The abdomen was therefore closed. Postoperative duodenal drainage revealed only traces of tryptic activity and 0.6 per cent unit of lipase. The child passed pencil-sized fragments of what appeared to be coagulated protein. Microscopic appearance was reported as " a m o r p h o u s material with no structural p a t t e r n . " F a t stains showed only a small amount of fat. Chemical analysis revealed that this material contained 65.8 rag. of nitrogen per gram (427.7 rag. of protein per gram), 66 rag. of fat per gram, 15.5 rag. of calcium per gram, 0.15 mEq. of sodium per gram, and .065 mEq. of potassium per gram ( d r y weight). The low potassium compared TABLE I.
SWEAT TESTS
TWIN A .. TWIN B AGE ~ CHLOCIILO(MONTHS) SODIUM RINE SODIUM RINE 61.0 90 88 72 92.3 109 150 180 133.0 127 178 172 Duodenal D~'ainage No trypsin No trypsin
with the sodium content suggests that this material at I1o time had the potassium-sodium ratio of 2:2 which is characteristic of living tissue. The protein composition was similar to that of evaporated milk. The child remained obstructed in spite of the instillation of pancreatic granules through a duodenal tube. A reopening of the laparotomy was therefore performed. The abnormal intestinal contents were forcibly removed from the terminal ileum through an ileostomy and a decompression ileostomy tube was left in place. Except for a transient episode of hypokalemia, the postoperative course was uneventful. Although there was no doubt that these children had pancreatic cystic fibrosis, both of them have done v e r y well with only minor gastrointestinal and respiratory symptoms. The diag-
8]5
nosis was, in fact, questioned and the sweat tests were repeated at another institution when the twins were 1 year old. The tests were again characteristic of this disorder. SUMMARY
1. A case of intestinal obstruction from a milk protein east of the entire intestine is reported. 2. Although this is believed to be the first such report, its actual ineidence in milder form in normal children as well as in infants with pancreatic fibrosis is open to speculation. 3. The latter part of this narrative also illustrates h o w asymptomatie cystic fibrosis of the pancreas may exist even when parents are aware of the diagnosis and are apprehensively searching for symptoms. REFERENCES ]. Shwachman, I-I., Pryles, C. V., and Gross, R. E.: Meconium Ileus: Clinical Study of 20 Surviving Patients, A. M. "A. J. Dis. Child. 91: 223, 1956. 2. Falls, F. H., and Jaffe, R. It.: Intestinal Obstruction in the Newborn Due to Mucus Plug, Am. J. Obst. & Gynec. 22: 409, 1931. 3. Emery, J. L.: Ab1{ormalities in Meconium of the Foetus and Newborn, Arch. Dis. Childhood 32: 17, 1957. 4. Zachary, R. ]3.: Meconium and Faecal Plugs in the Newborn, Arch. ])is. Childhood 32: 22, 1957. 5. Rogers, J. D., Baggenstoss, A. M., MusGrove, J. E., and Kennedy, R. L. J.: Intestinal Obstruction Associated With Fibrocystic Disease of the Pancreas, Minnesota Med. 34: 1075, 195l. 6. Levy, E.: A Case of Fibrocystic Disease of the Pancreas With Intestinal Obstruction, Arch. Dis. Childhood 26: 335, 1951. 7. Fisher, O. D.: Intestinal Obstruction as Late Complication of Fibrocystic Disease of Pa~icreas (Mueosis), Arch. ])is. Childhood 29: 262, 1954. 8. Nixon~ It. H.: Inspissation Obstruction of the Small Intestine in a Survivor From Meeonium Ileus, Great Ormond St. J. (no. 9), p. 43, 1955. 9. ]3irse, E. L.: Intestinal Obstruction as a Late Complication of Fibrocystic Disease of the Pancreas, Brit. M. J. 2: 286, 1956. 10. Andersen, D. H.: Cystic Fibrosis of the Pt~ncreas, J. Chron. Dis. 7: 58, 1958. 11. Shwachman, It.: Year ]3ook of Pediatrics, 1955-56, p. 203.