Congenital Colonic Atresia and Stenosis
Medad Schiller, MD, Jerusalem, Israel lthamar Aviad, MD, Jerusalem, Israel Herbert Freund, MD, Jerusalem, Israel
The incidence of congenital atresia and stenosis of the intestine has been estimated to be from 1 in 20,000 births [I] to 1 in 1,500 births [2], with most cases occurring in the small bowel. Large bowel atresia or stenosis is rare, comprising only 1.8 to 15 per cent of the cases [3-51, and colonk stenosis is much rarer than atresia. Up to 1970, 72 cases of congenital atresia or stenosis of the colon were reported [3,6], and since 1970 only 16 more cases were added to the literature [ 7,8]. Five successfully diagnosed and treated infants, two with atresia and three with stenosis of the colon, are the subject of this report. Case Reports Case 1. In a 2,600 g newborn female infant, marked abdominal distension and vigorous vomiting developed at age 36 hours. On physical examination visible and audible hyperperistaltic waves were observed. The plain film of the abdomen showed low intestinal obstruction with a “ground glass” appearance in the right lower abdomen. Barium enema disclosed an unused left hemicolon. The contrast material could not be passed beyond the mid-transverse colon, where a blind end was demonstrated (Figure 1). On exploration a large blind-ending distended ascending colon was found with the appendix entering the cecum (Figure 2). There was a V-shaped defect of the mesentery, and the small blind end of the distal colon was noted. Because of the poor general condition of the infant, no attempt was made to perform an anastomosis, and the proximal atretic ascending colon was brought out as an end colostomy. At age 3 months, because of repeated prolapse of the colostomy, reoperation was performed; the right colon and the blind end of the transverse colon were resected and an From the Departments of Pediatric Surgery and Radiology, Hadassah University Hospitals, Jerusalem. Israel. Reprint requests should be addressed to Medad Schiller, MD, Department of Pediatric Surgery, Hadassah University Medical Center, Ein Karem, Jerusalem, Israel.
Volume 139, November 1979
end-to-side ileotransversostomy was performed. Five years after the restoration of colonic continuity, the girl is developing in a normal fashion with no gastrointestinal problems. Case 2. A 48 hour old full-term (3,450 g) female infant was transferred to the pediatric surgical service because of abdominal distension, bilious vomiting, and failure to pass meconium. The physical examination and plain abdominal roentgenograms were consistent with low intestinal obstruction. Barium enema revealed an unused rectosigmoid that ended as a blind pouch (Figure 3). After preparation of the infant, exploratory surgery was performed and a descending colonic atresia found. There was no continuity between the two atretic segments, the mesentery being intact. A descending loop colostomy was performed. At age 6 months the infant was reoperated on, the colostomy together with the two atretic segments were resected, and end-to-end colocolostomy was performed. Eleven years after this operation the child is normal. Case 3. A full-term (3,100 g) male infant, the fourth child in the family, was born after an uncomplicated pregnancy and delivery and was transferred at age 3 days to the pediatric surgical service because of a distended abdomen, bilious vomiting, and failure to pass meconium. On physical examination the infant was dehydrated and marked abdominal distension, with audible high-pitched peristalsis, was noted. Bilateral club foot was also observed. On rectal examination the examining finger could not be passed beyond 2 to 2.5 cm from the anocutaneous junction. Plain films of the abdomen revealed multiple distended bowel loops. On barium enema only a small blind-ending rectal pouch could be filled. After rehydration and gastric decompression, the infant was operated on. A distended descending colon was found continuing toward the rectum as a thin (3 mm in diameter) hypoplastic sigmoid loop. A descending loop colostomy was performed and the infant had an uneventful recovery and developed well until age 6 months, when a right inguinal hernia was repaired because of repeated incarcerations. During this hospitalization barium enemas from below and from the distal co-
721
Schiller et al
Figure 1, left. Case 1. Barium enema shows fitting of an unused left hemicolon ending blindly in the mid-transverse colon. /Vote the marked dilatation of the right hemicolon and small bowel. Figure 2, right. Case 1. Atretic ascending coton found at operation. Note the cewm and appendix at the bottom.
lostomy were performed, and a marked stenotic sigmoid colon was demonstrated (Figures 4 and 5). Up to age 15 months the blind rectal pouch was digitally dilated and elongated by mother, reaching a length of 4.5 cm. At this stage reoperation was performed, the stenotic sigmoid measuring 9.5 cm was resected, and an anterior anastomosis between the descending colon and rectal pouch accomplished. The previous colostomy was left in place to protect the very low placed colonic anastomosis. Three months later, after a distal barium enema that showed good passage through the anastomosis and excellent emptying of the distal bowel, the colostomy was resected and colonic continuity restored by an end-to-end colocolostomy. Four and a half years after surgery, the patient has normal bowel movements. The bilateral club foot was also corrected and the boy is walking without difficulty.
Figure 3. Case 2. Barium enema shows filling of an unused rectosigmoid ending as a blind loop. 722
Case 4. A male infant weighing 3,600 g, born after a 32 week gestation by cesarean section to a diabetic mother, was admitted on the second day of life with a history of increasing abdominal distension and vomiting. A plain abdominal film revealed free air under the diaphragm. At operation a free perforation was found in the middle of a gangrenous jejunal loop. The perforation seemed to be the result of vascular interference due to segmental volvulus. Resection of 25 cm of jejunum and primary anastomosis were performed. Exploration of the rest of the abdomen disclosed that the right and transverse colon were dilated The American Journal 01 Surgery
Congenital Colonic Atresia and Stenosis
Figure 4, left. Case 3. Barium enema shows well demonstrated rectum with marked narrowing of the sigmotd colon. Figure 5, right. Case 3. Barium enema shows fflllng of distal colostomy, demonstrating a normal distal descending colon with severe stenosis of the sigmoid.
up to the splenic flexure, where the colon turned out to be very narrow, as if atretic. A right transverse loop colostomy was performed. The postoperative course was uneventful and the child did well. A barium enema examination performed at age 6 months demonstrated a very narrow area of stenosis at the left transverse colon and splenic flexure, measuring 3 to 4 cm in length. The area of stenosis was resected together with the colostomy site, and end-to-end anastomosis was accomplished. At age II/s years, the child has normal bowel movements and is thriving. Case 5. A 1 day old male infant weighing 2,900 g, born after an uncomplicated pregnancy and delivery, was admitted to the department of pediatric surgery because of marked abdominal distension and bilious vomiting. Before admission the infant passed two small meconial stools. On physical examination he was alert, with no sign of sepsis. The abdomen was markedly distended, with visible and audible hyperperistaltic waves. On rectal examination the examining finger could not be passed beyond a point 2.5 to 3 cm from the anocutaneous junction. Plain abdominal roentgenograms revealed low intestinal obstruction, and a barium enema showed a marked 4 cm stenotic segment of the upper rectum and lower sigmoid. The proximal colon was markedly distended. At operation, a descending loop colostomy was performed. On exploration a stenotic large bowel extending 2.5 cm above the peritoneal reflexion was observed. Six months after colostomy, the infant is awaiting further surgery at a later stage.
Comments The dilemma of the intestine
of the etiology of atresia
and stenosis
in the newborn, which has been the
Volume 139, November 1979
subject of much discussion and debate, seems to be settled now, with most authors accepting the theory of intrauterine injury to the intestine due to interference with the mesenteric blood supply [9]. The grade of interference, which might be caused by either thrombosis or embolization, is responsible for the end result. The more extensive forms of atresia result from complete obstruction of the mesentric vessels, whereas stenosis probably results from incomplete obstruction. The recently recognized acquired colonic stenosis after necrotizing entercolitis provides additional support for vascular interference as the cause of stenosis of the large intestine (IO12).
Infants with colonic atresia or stenosis usually present within 24 to 48 hours after birth with symptoms of low intestinal obstruction, distension, failure to pass meconium, and vomiting. A flat and upright plain abdominal radiograph offers some information, but the diagnosis can be made only by the use of a barium enema to demonstrate a small unused distal colon ending blindly. Because the mortality in nonoperated patients is 100 per cent [12], colonic atresia and stenosis are surgical emergencies for which early diagnosis and exploration of the abdomen should be performed, after a short and intensive preparation with nasogastric suctioning and intravenous administration of fluids, plasma preparations, and antibiotics. Operative management must include thorough exploration of the abdomen, adequate decompression of the obstructed bowel, resection of the dilated 723
Schiller et al
preatretic part, and resumption of bowel continuity. In all of our patients we elected to use a two-stage approach. The first stage included exploration of the abdomen and the performance of a colostomy in a healthy bowel proximal to the atresia or stenosis. This procedure is technically simple, safe, and efficient in immediate bowel depression and is in our opinion the best method of management for these very sick infants with obstruction. A few months later, when the infant is thriving, intestinal continuity is reestablished by resection of the involved bowel and end-to-end anastomosis. This two-stage procedure, which guarantees safety for the affected infants, is accepted by some authors [7,13], while others advocate routine resection of the proximal dilated colon and primary anastomosis [4,9] or resection of the proximal colon with primary ileocolic anastomosis in atresias proximal to the splenic flexure and a two-stage procedure for more distal ones [3,14,15]. The importance of a thorough abdominal exploration is demonstrated clearly by patient 4, who suffered a neonatal jejunal perforation coexistent with a colon stenosis. Benson et al [3] reported three patients with concomitant small and large bowel atresia. Wilson et al [16] claims that 80 per cent of infants with colonic atresia have associated gastrointestinal anomalies, particularly rotation and fixation anomalies [16]. Overlooking colonic atresia or stenosis while operating for an other intestinal anomaly could result in a fatal outcome, as occurred in some of the patients of Benson et al [3]. ,Summary
Five more cases are added to the 88 reported cases of successfully treated newborns with congenital colonic atresia and stenosis. Because colonic atresia
724
and stenosis are lethal conditions when untreated, early diagnosis and operative treatment are major requisites for survival. A two-stage procedure consisting of an emergency colostomy for decompression as the first stage and an elective resection with anastomosis a few months later is recommended. The need for thorough exploration of the abdomen is emphasized because atresias may be multiple or may be associated with additional gastrointestinal anomalies. References 1. Webb CH, Wangensteen OH: Congenital intestinal atresia. Am JDis Child41: 262, 1931. 2. Evans CH: Atresias of the gastrointestinal tract. Sufg Gynecd Obstet92: 1, 1951. 3. Benson CD, Lofti MW, Brough AJ: Congenital atresia and stenosis of the colon. J fediafr Surg 3: 253, 1966. 4. Freeman NV: Congenital stenosis and atresia of the colon. Br J Surg 53: 595, 1966. 5. Lynn HB, Espinas EE: Intestinal atresia. Arch Surg 79: 357, 1959. 6. Erskine JM: Colonic stenosis in the newborn. J Pediatr Surg 5: 321, 1970. 7. Boles ET, Vossy LE, Ralston M: Atresia of the colon. J Pediafr Surg 11: 69, 1976. 6. Meradji M, Molenaar JC: Kongenitale Atresie des Kolon. Z Kinderchirurgie 18: 170, 1976. 9. Louw JH: Congenital intestinal atresia and stenosis. Ann R Co/l Surg Engl59: 458, 1959. 10. Chiba T, Wantabe I, Kasai M: Colonic atresia following necrotizing entercolitis. J Pediatr Surg 10: 965, 1975. 11. Krasna IM, Becker JM, Schneider KM, Beck AR: Colonic stenosis following necrotizing entercolitis of the newborn. J Pediatr Surg 5: 2001, 1974. 12. Stein H, Kavin I, Faerber EN: Colonic strictures following necrotizing entercolitis. J Pediatf Surg 10: 943, 1975. 13. Hartman SW, Kinkanoon WN, Greaney EM: Congenital atresia of the colon. Am Surg 19: 699, 1963. 14. Coran AG, Eraklis AJ: Atresia of the colon. Surgery 65: 828, 1969. 15. Sturim HS, Ternberg JL: Congenital atresia of the colon. Surgery 59: 458, 1966. 16. Wilson BJ, Nelson A, Harshbarger M: Congenital atresia of the colon. Surg Gynecol Obstet 99: 34, 1954.
The American Journal of Surgery
Medad Schiller, MD, Jerusalem, Israel lthamar Aviad, MD, Jerusalem, Israel Herbert Freund, MD, Jerusalem, Israel
The incidence of congenital atresia and stenosis of the intestine has been estimated to be from 1 in 20,000 births [I] to 1 in 1,500 births [2], with most cases occurring in the small bowel. Large bowel atresia or stenosis is rare, comprising only 1.8 to 15 per cent of the cases [3-51, and colonk stenosis is much rarer than atresia. Up to 1970, 72 cases of congenital atresia or stenosis of the colon were reported [3,6], and since 1970 only 16 more cases were added to the literature [ 7,8]. Five successfully diagnosed and treated infants, two with atresia and three with stenosis of the colon, are the subject of this report. Case Reports Case 1. In a 2,600 g newborn female infant, marked abdominal distension and vigorous vomiting developed at age 36 hours. On physical examination visible and audible hyperperistaltic waves were observed. The plain film of the abdomen showed low intestinal obstruction with a “ground glass” appearance in the right lower abdomen. Barium enema disclosed an unused left hemicolon. The contrast material could not be passed beyond the mid-transverse colon, where a blind end was demonstrated (Figure 1). On exploration a large blind-ending distended ascending colon was found with the appendix entering the cecum (Figure 2). There was a V-shaped defect of the mesentery, and the small blind end of the distal colon was noted. Because of the poor general condition of the infant, no attempt was made to perform an anastomosis, and the proximal atretic ascending colon was brought out as an end colostomy. At age 3 months, because of repeated prolapse of the colostomy, reoperation was performed; the right colon and the blind end of the transverse colon were resected and an From the Departments of Pediatric Surgery and Radiology, Hadassah University Hospitals, Jerusalem. Israel. Reprint requests should be addressed to Medad Schiller, MD, Department of Pediatric Surgery, Hadassah University Medical Center, Ein Karem, Jerusalem, Israel.
Volume 139, November 1979
end-to-side ileotransversostomy was performed. Five years after the restoration of colonic continuity, the girl is developing in a normal fashion with no gastrointestinal problems. Case 2. A 48 hour old full-term (3,450 g) female infant was transferred to the pediatric surgical service because of abdominal distension, bilious vomiting, and failure to pass meconium. The physical examination and plain abdominal roentgenograms were consistent with low intestinal obstruction. Barium enema revealed an unused rectosigmoid that ended as a blind pouch (Figure 3). After preparation of the infant, exploratory surgery was performed and a descending colonic atresia found. There was no continuity between the two atretic segments, the mesentery being intact. A descending loop colostomy was performed. At age 6 months the infant was reoperated on, the colostomy together with the two atretic segments were resected, and end-to-end colocolostomy was performed. Eleven years after this operation the child is normal. Case 3. A full-term (3,100 g) male infant, the fourth child in the family, was born after an uncomplicated pregnancy and delivery and was transferred at age 3 days to the pediatric surgical service because of a distended abdomen, bilious vomiting, and failure to pass meconium. On physical examination the infant was dehydrated and marked abdominal distension, with audible high-pitched peristalsis, was noted. Bilateral club foot was also observed. On rectal examination the examining finger could not be passed beyond 2 to 2.5 cm from the anocutaneous junction. Plain films of the abdomen revealed multiple distended bowel loops. On barium enema only a small blind-ending rectal pouch could be filled. After rehydration and gastric decompression, the infant was operated on. A distended descending colon was found continuing toward the rectum as a thin (3 mm in diameter) hypoplastic sigmoid loop. A descending loop colostomy was performed and the infant had an uneventful recovery and developed well until age 6 months, when a right inguinal hernia was repaired because of repeated incarcerations. During this hospitalization barium enemas from below and from the distal co-
721
Schiller et al
Figure 1, left. Case 1. Barium enema shows fitting of an unused left hemicolon ending blindly in the mid-transverse colon. /Vote the marked dilatation of the right hemicolon and small bowel. Figure 2, right. Case 1. Atretic ascending coton found at operation. Note the cewm and appendix at the bottom.
lostomy were performed, and a marked stenotic sigmoid colon was demonstrated (Figures 4 and 5). Up to age 15 months the blind rectal pouch was digitally dilated and elongated by mother, reaching a length of 4.5 cm. At this stage reoperation was performed, the stenotic sigmoid measuring 9.5 cm was resected, and an anterior anastomosis between the descending colon and rectal pouch accomplished. The previous colostomy was left in place to protect the very low placed colonic anastomosis. Three months later, after a distal barium enema that showed good passage through the anastomosis and excellent emptying of the distal bowel, the colostomy was resected and colonic continuity restored by an end-to-end colocolostomy. Four and a half years after surgery, the patient has normal bowel movements. The bilateral club foot was also corrected and the boy is walking without difficulty.
Figure 3. Case 2. Barium enema shows filling of an unused rectosigmoid ending as a blind loop. 722
Case 4. A male infant weighing 3,600 g, born after a 32 week gestation by cesarean section to a diabetic mother, was admitted on the second day of life with a history of increasing abdominal distension and vomiting. A plain abdominal film revealed free air under the diaphragm. At operation a free perforation was found in the middle of a gangrenous jejunal loop. The perforation seemed to be the result of vascular interference due to segmental volvulus. Resection of 25 cm of jejunum and primary anastomosis were performed. Exploration of the rest of the abdomen disclosed that the right and transverse colon were dilated The American Journal 01 Surgery
Congenital Colonic Atresia and Stenosis
Figure 4, left. Case 3. Barium enema shows well demonstrated rectum with marked narrowing of the sigmotd colon. Figure 5, right. Case 3. Barium enema shows fflllng of distal colostomy, demonstrating a normal distal descending colon with severe stenosis of the sigmoid.
up to the splenic flexure, where the colon turned out to be very narrow, as if atretic. A right transverse loop colostomy was performed. The postoperative course was uneventful and the child did well. A barium enema examination performed at age 6 months demonstrated a very narrow area of stenosis at the left transverse colon and splenic flexure, measuring 3 to 4 cm in length. The area of stenosis was resected together with the colostomy site, and end-to-end anastomosis was accomplished. At age II/s years, the child has normal bowel movements and is thriving. Case 5. A 1 day old male infant weighing 2,900 g, born after an uncomplicated pregnancy and delivery, was admitted to the department of pediatric surgery because of marked abdominal distension and bilious vomiting. Before admission the infant passed two small meconial stools. On physical examination he was alert, with no sign of sepsis. The abdomen was markedly distended, with visible and audible hyperperistaltic waves. On rectal examination the examining finger could not be passed beyond a point 2.5 to 3 cm from the anocutaneous junction. Plain abdominal roentgenograms revealed low intestinal obstruction, and a barium enema showed a marked 4 cm stenotic segment of the upper rectum and lower sigmoid. The proximal colon was markedly distended. At operation, a descending loop colostomy was performed. On exploration a stenotic large bowel extending 2.5 cm above the peritoneal reflexion was observed. Six months after colostomy, the infant is awaiting further surgery at a later stage.
Comments The dilemma of the intestine
of the etiology of atresia
and stenosis
in the newborn, which has been the
Volume 139, November 1979
subject of much discussion and debate, seems to be settled now, with most authors accepting the theory of intrauterine injury to the intestine due to interference with the mesenteric blood supply [9]. The grade of interference, which might be caused by either thrombosis or embolization, is responsible for the end result. The more extensive forms of atresia result from complete obstruction of the mesentric vessels, whereas stenosis probably results from incomplete obstruction. The recently recognized acquired colonic stenosis after necrotizing entercolitis provides additional support for vascular interference as the cause of stenosis of the large intestine (IO12).
Infants with colonic atresia or stenosis usually present within 24 to 48 hours after birth with symptoms of low intestinal obstruction, distension, failure to pass meconium, and vomiting. A flat and upright plain abdominal radiograph offers some information, but the diagnosis can be made only by the use of a barium enema to demonstrate a small unused distal colon ending blindly. Because the mortality in nonoperated patients is 100 per cent [12], colonic atresia and stenosis are surgical emergencies for which early diagnosis and exploration of the abdomen should be performed, after a short and intensive preparation with nasogastric suctioning and intravenous administration of fluids, plasma preparations, and antibiotics. Operative management must include thorough exploration of the abdomen, adequate decompression of the obstructed bowel, resection of the dilated 723
Schiller et al
preatretic part, and resumption of bowel continuity. In all of our patients we elected to use a two-stage approach. The first stage included exploration of the abdomen and the performance of a colostomy in a healthy bowel proximal to the atresia or stenosis. This procedure is technically simple, safe, and efficient in immediate bowel depression and is in our opinion the best method of management for these very sick infants with obstruction. A few months later, when the infant is thriving, intestinal continuity is reestablished by resection of the involved bowel and end-to-end anastomosis. This two-stage procedure, which guarantees safety for the affected infants, is accepted by some authors [7,13], while others advocate routine resection of the proximal dilated colon and primary anastomosis [4,9] or resection of the proximal colon with primary ileocolic anastomosis in atresias proximal to the splenic flexure and a two-stage procedure for more distal ones [3,14,15]. The importance of a thorough abdominal exploration is demonstrated clearly by patient 4, who suffered a neonatal jejunal perforation coexistent with a colon stenosis. Benson et al [3] reported three patients with concomitant small and large bowel atresia. Wilson et al [16] claims that 80 per cent of infants with colonic atresia have associated gastrointestinal anomalies, particularly rotation and fixation anomalies [16]. Overlooking colonic atresia or stenosis while operating for an other intestinal anomaly could result in a fatal outcome, as occurred in some of the patients of Benson et al [3]. ,Summary
Five more cases are added to the 88 reported cases of successfully treated newborns with congenital colonic atresia and stenosis. Because colonic atresia
724
and stenosis are lethal conditions when untreated, early diagnosis and operative treatment are major requisites for survival. A two-stage procedure consisting of an emergency colostomy for decompression as the first stage and an elective resection with anastomosis a few months later is recommended. The need for thorough exploration of the abdomen is emphasized because atresias may be multiple or may be associated with additional gastrointestinal anomalies. References 1. Webb CH, Wangensteen OH: Congenital intestinal atresia. Am JDis Child41: 262, 1931. 2. Evans CH: Atresias of the gastrointestinal tract. Sufg Gynecd Obstet92: 1, 1951. 3. Benson CD, Lofti MW, Brough AJ: Congenital atresia and stenosis of the colon. J fediafr Surg 3: 253, 1966. 4. Freeman NV: Congenital stenosis and atresia of the colon. Br J Surg 53: 595, 1966. 5. Lynn HB, Espinas EE: Intestinal atresia. Arch Surg 79: 357, 1959. 6. Erskine JM: Colonic stenosis in the newborn. J Pediatr Surg 5: 321, 1970. 7. Boles ET, Vossy LE, Ralston M: Atresia of the colon. J Pediafr Surg 11: 69, 1976. 6. Meradji M, Molenaar JC: Kongenitale Atresie des Kolon. Z Kinderchirurgie 18: 170, 1976. 9. Louw JH: Congenital intestinal atresia and stenosis. Ann R Co/l Surg Engl59: 458, 1959. 10. Chiba T, Wantabe I, Kasai M: Colonic atresia following necrotizing entercolitis. J Pediatr Surg 10: 965, 1975. 11. Krasna IM, Becker JM, Schneider KM, Beck AR: Colonic stenosis following necrotizing entercolitis of the newborn. J Pediatr Surg 5: 2001, 1974. 12. Stein H, Kavin I, Faerber EN: Colonic strictures following necrotizing entercolitis. J Pediatf Surg 10: 943, 1975. 13. Hartman SW, Kinkanoon WN, Greaney EM: Congenital atresia of the colon. Am Surg 19: 699, 1963. 14. Coran AG, Eraklis AJ: Atresia of the colon. Surgery 65: 828, 1969. 15. Sturim HS, Ternberg JL: Congenital atresia of the colon. Surgery 59: 458, 1966. 16. Wilson BJ, Nelson A, Harshbarger M: Congenital atresia of the colon. Surg Gynecol Obstet 99: 34, 1954.
The American Journal of Surgery
